Salivary gland tumorsIntroduction

Tumors of the salivary glands are uncommon and represent 2-4 of head and neck neoplasms They may be broadly categorized into benign neoplasms tumor-like conditions and malignant neoplasms The glands are divided into major and minor salivary gland categories The major salivary glands are the parotid the submandibular and the sublingual glands The minor glands are dispersed throughout the upper aerodigestive submucosa (ie palate lip pharynx nasopharynx larynx parapharyngeal space)

Most (70) salivary gland tumors (SGTs) originate in the parotid gland The remaining tumors arise in the submandibular gland (8) and minor salivary glands (22) Although 75 of parotid gland tumors are benign slightly more than 50 of tumors of the submandibular gland and 80 of minor SGTs are found to be malignant Pleomorphic adenomas (benign mixed tumors) are the most common benign SGTs comprising 85 of all salivary gland neoplasms

The ubiquitous deposition of the minor salivary glands complicates the diagnosis and management of SGTs The approach for a suspected tumor of the minor salivary glands begins with a thorough history and a physical examination Radiographic imaging (CT with or without MRI) and a histopathologic diagnosis (obtained based on fine needle aspiration biopsy [FNAB]) often provide useful information prior to definitive surgical therapy

CLASSIFICATIONS1 Adenomas1 Pleomorphic adenoma2 Myoepithelioma (myoepithelial adenoma)3 Basal cell adenoma4 Warthinrsquos tumor (adenolymphoma)5 Oncocytoma (Oncocytic adenoma)6 Canalicular adenoma7 Sebaceous adenoma8 Ductal Papilloma

Inverted ductal papilloma Intraductal papilloma Sialadenoma papilliferum

1

9 Cystadenoma Papillary cystadenoma Mucinous cystadenoma

2 Carcinomas1 Acinic cell carcinoma2 Mucoepidermoid carcinoma3 Adenoid cystic carcinoma4 Polymorphous low-grade adenocarcinoma5 Epithelial-myoepithelial carcinoma6 Basal cell adenocarcinoma7 Sebaceous carcinoma8 Papillary cystadenocarcinoma9 Mucinous adenocarcinoma10 Oncocytic carcinoma11 Salivary duct carcinoma12 Adenocarcinoma13 Malignant myoepithelioma14 Squamous cell carcinoma15 Small cell carcinoma16 Undifferentiated carcinoma

3 Miscellaneous 1 Nonepithelial tumors2 Malignant lymphomas3 Secondary tumors4 Unclassified tumors5 Tumor-like lesions

Siladenosis Oncocytosis Necrotizing sialometaplasia (salivary gland infarction) Benign lymphoepithelial lesion Salivary gland cysts Chronic sclerosing sialedenitis of submandibular gland (Kuttner tumor) Cystic lymphoid hyperplasia in patients with acquired immunodeficiency

syndrome

INCIDENCESalivary gland tumors hellip3-4 of all head amp neck neoplasm70 originate in parotid glandgt 70of them are Benign70 of them hellipplemorphic adenoma

2

10 of parotid tumorshellip mucoepidermoid carcinoma

3

4

PathophysiologyI Multi-cellular theory Salivary gland arise from the adult differentiated counter part of salivary glandunit10487081048708 Acinar cell acinous tumor10487081048708 Striated duct cell oncocytic tumor10487081048708 Intercalated duct cell mixed tumor10487081048708 Excretory duct cells mucoepidermoid carcinoma amp squamous cell carcinoma

5

II Bicellular Theory The basal cell of excretory amp intercalated duct act as stem cells10487081048708 Intercalated duct stem cell acinous cell carcinoma adenoid cystic carcinoma mixed tumor amp oncocytic tumors10487081048708 Excretory duct stem cell squamous cell carcinoma amp mucoepidermoid carcinoma

AetiologyI Radiation Several studies have implicate radiation associated neoplasm Does-response pattern Mostly parotid Commonly mucoepidermoid carcinoma

II Epstein-Barr Virus The consistent association of E B V with lymphoepithelial carcinoma of the

salivary gland suggest the virus probably plays causal role Similar pathogenesis of nasopharyngeal carcinoma no evidence of a causal role of EBV in other primary tumors of the salivary gland

III Genetic FactorsMany genetic alternation may be responsible for increased likelihood of developing salivary gland neoplasm as allelic loss structural rearrangement monosomyamp polysomy

IV Other factors Silica dust or wood dust Using Kerosene as cooking fuel Warthinrsquos tumor is strongly associated with cigarette smoking

DiagnosisI Clinical features

Usually present as slowly growing painless swelling A sudden increase in size ( infection cystic degeneration hemorrhage inside the

mass or malignant transformation) malignant indicators asFacial nerve paresis or paralysis Weakness or numbness of

the tongue or in distribution of branches of trigeminal nerve Pain Fixation Cervical adenopathy

History of malignant skin lesion should be clarified as case of metastatic disease to the parotidNeed full examination especially

6

The mass itself Skin of face or scalp Oropharynx C N S Cervical lymph node

II Fine needle aspiration biopsyEfficacy is well established

Accuracy = 84-97 Sensitivity = 54-95 Specificity = 86=100

Safe well tolerated

Opponents

7

Doesnrsquot change management Surgery regardless of reported diagnosis Obscuring final pathologic diagnosis Frequency of ldquoinadequaterdquo sampling requires multiple biopsies prolongs course until definitive treatment increases cost

Proponent Important to distinguish benign vs malignant nature of neoplasm Preoperative patient counseling Surgical planning Differentiate between neoplastic and non-neoplastic processes Avoid surgery in large number of patients

III Radiology Conventional radiography amp sialography are rarely used C T amp M R I give better understanding

o Location amp extent of the tumoro Its relation to major neurovascular structureo Perineural spreado Skull base invasiono Intracranial extensiono But most of the time canrsquot be differentiate between benign amp malignant

entities Nuclear imaging using Technetium 99m pertechnetate is rarely obtained because FNAB may provide better information Other imaging studies Ultrasonography Color doppler sonography

IV Biopsies-Incisional intraoral biopsy of parapharyngeal tumor should not be performed-The proper approach to parotid neoplasm is parotidectomy which diagnostic amp curative- Open biopsy done rarely amp in the patient who not surgical candidate amp for whom FNAB is not diagnostic

Benign tumorsPleomorphic Adenoma

8

Most common of all salivary gland neoplasms 70 of parotid tumors 4th-6th decades FM = 3-41 Slow-growing painlessfirm mass Rarely progresive

1048708 Gross pathology Smooth Well-demarcated Solid Cystic changes Myxoid stroma

1048708 Histology 1048708 Mixture of epithelial myopeithelial and stromal components 1048708 No true capsule 1048708 Tumor pseudopods

Warthinrsquos Tumor papillary cystadenoma lymphomatosum 6-10 of parotid neoplasms Older Caucasian males 10 bilateral or multicentric 3 with associated neoplasms Presentation slow-growing painless mass

Gross pathology Encapsulated Smoothlobulated surface Cystic spaces of variable size with viscous fluid shaggy epithelium Solid areas with white nodules representing lymphoid follicles

Histology1048708Papillary projections into cystic spaces surrounded by lymphoid stroma

Oncocytoma lt 1of salivary gand tumors Parotid is the most frequent site Slow-growing painless mass 6th decade

9

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

9 Cystadenoma Papillary cystadenoma Mucinous cystadenoma

2 Carcinomas1 Acinic cell carcinoma2 Mucoepidermoid carcinoma3 Adenoid cystic carcinoma4 Polymorphous low-grade adenocarcinoma5 Epithelial-myoepithelial carcinoma6 Basal cell adenocarcinoma7 Sebaceous carcinoma8 Papillary cystadenocarcinoma9 Mucinous adenocarcinoma10 Oncocytic carcinoma11 Salivary duct carcinoma12 Adenocarcinoma13 Malignant myoepithelioma14 Squamous cell carcinoma15 Small cell carcinoma16 Undifferentiated carcinoma

3 Miscellaneous 1 Nonepithelial tumors2 Malignant lymphomas3 Secondary tumors4 Unclassified tumors5 Tumor-like lesions

Siladenosis Oncocytosis Necrotizing sialometaplasia (salivary gland infarction) Benign lymphoepithelial lesion Salivary gland cysts Chronic sclerosing sialedenitis of submandibular gland (Kuttner tumor) Cystic lymphoid hyperplasia in patients with acquired immunodeficiency

syndrome

INCIDENCESalivary gland tumors hellip3-4 of all head amp neck neoplasm70 originate in parotid glandgt 70of them are Benign70 of them hellipplemorphic adenoma

2

10 of parotid tumorshellip mucoepidermoid carcinoma

3

4

PathophysiologyI Multi-cellular theory Salivary gland arise from the adult differentiated counter part of salivary glandunit10487081048708 Acinar cell acinous tumor10487081048708 Striated duct cell oncocytic tumor10487081048708 Intercalated duct cell mixed tumor10487081048708 Excretory duct cells mucoepidermoid carcinoma amp squamous cell carcinoma

5

II Bicellular Theory The basal cell of excretory amp intercalated duct act as stem cells10487081048708 Intercalated duct stem cell acinous cell carcinoma adenoid cystic carcinoma mixed tumor amp oncocytic tumors10487081048708 Excretory duct stem cell squamous cell carcinoma amp mucoepidermoid carcinoma

AetiologyI Radiation Several studies have implicate radiation associated neoplasm Does-response pattern Mostly parotid Commonly mucoepidermoid carcinoma

II Epstein-Barr Virus The consistent association of E B V with lymphoepithelial carcinoma of the

salivary gland suggest the virus probably plays causal role Similar pathogenesis of nasopharyngeal carcinoma no evidence of a causal role of EBV in other primary tumors of the salivary gland

III Genetic FactorsMany genetic alternation may be responsible for increased likelihood of developing salivary gland neoplasm as allelic loss structural rearrangement monosomyamp polysomy

IV Other factors Silica dust or wood dust Using Kerosene as cooking fuel Warthinrsquos tumor is strongly associated with cigarette smoking

DiagnosisI Clinical features

Usually present as slowly growing painless swelling A sudden increase in size ( infection cystic degeneration hemorrhage inside the

mass or malignant transformation) malignant indicators asFacial nerve paresis or paralysis Weakness or numbness of

the tongue or in distribution of branches of trigeminal nerve Pain Fixation Cervical adenopathy

History of malignant skin lesion should be clarified as case of metastatic disease to the parotidNeed full examination especially

6

The mass itself Skin of face or scalp Oropharynx C N S Cervical lymph node

II Fine needle aspiration biopsyEfficacy is well established

Accuracy = 84-97 Sensitivity = 54-95 Specificity = 86=100

Safe well tolerated

Opponents

7

Doesnrsquot change management Surgery regardless of reported diagnosis Obscuring final pathologic diagnosis Frequency of ldquoinadequaterdquo sampling requires multiple biopsies prolongs course until definitive treatment increases cost

Proponent Important to distinguish benign vs malignant nature of neoplasm Preoperative patient counseling Surgical planning Differentiate between neoplastic and non-neoplastic processes Avoid surgery in large number of patients

III Radiology Conventional radiography amp sialography are rarely used C T amp M R I give better understanding

o Location amp extent of the tumoro Its relation to major neurovascular structureo Perineural spreado Skull base invasiono Intracranial extensiono But most of the time canrsquot be differentiate between benign amp malignant

entities Nuclear imaging using Technetium 99m pertechnetate is rarely obtained because FNAB may provide better information Other imaging studies Ultrasonography Color doppler sonography

IV Biopsies-Incisional intraoral biopsy of parapharyngeal tumor should not be performed-The proper approach to parotid neoplasm is parotidectomy which diagnostic amp curative- Open biopsy done rarely amp in the patient who not surgical candidate amp for whom FNAB is not diagnostic

Benign tumorsPleomorphic Adenoma

8

Most common of all salivary gland neoplasms 70 of parotid tumors 4th-6th decades FM = 3-41 Slow-growing painlessfirm mass Rarely progresive

1048708 Gross pathology Smooth Well-demarcated Solid Cystic changes Myxoid stroma

1048708 Histology 1048708 Mixture of epithelial myopeithelial and stromal components 1048708 No true capsule 1048708 Tumor pseudopods

Warthinrsquos Tumor papillary cystadenoma lymphomatosum 6-10 of parotid neoplasms Older Caucasian males 10 bilateral or multicentric 3 with associated neoplasms Presentation slow-growing painless mass

Gross pathology Encapsulated Smoothlobulated surface Cystic spaces of variable size with viscous fluid shaggy epithelium Solid areas with white nodules representing lymphoid follicles

Histology1048708Papillary projections into cystic spaces surrounded by lymphoid stroma

Oncocytoma lt 1of salivary gand tumors Parotid is the most frequent site Slow-growing painless mass 6th decade

9

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

10 of parotid tumorshellip mucoepidermoid carcinoma

3

4

PathophysiologyI Multi-cellular theory Salivary gland arise from the adult differentiated counter part of salivary glandunit10487081048708 Acinar cell acinous tumor10487081048708 Striated duct cell oncocytic tumor10487081048708 Intercalated duct cell mixed tumor10487081048708 Excretory duct cells mucoepidermoid carcinoma amp squamous cell carcinoma

5

II Bicellular Theory The basal cell of excretory amp intercalated duct act as stem cells10487081048708 Intercalated duct stem cell acinous cell carcinoma adenoid cystic carcinoma mixed tumor amp oncocytic tumors10487081048708 Excretory duct stem cell squamous cell carcinoma amp mucoepidermoid carcinoma

AetiologyI Radiation Several studies have implicate radiation associated neoplasm Does-response pattern Mostly parotid Commonly mucoepidermoid carcinoma

II Epstein-Barr Virus The consistent association of E B V with lymphoepithelial carcinoma of the

salivary gland suggest the virus probably plays causal role Similar pathogenesis of nasopharyngeal carcinoma no evidence of a causal role of EBV in other primary tumors of the salivary gland

III Genetic FactorsMany genetic alternation may be responsible for increased likelihood of developing salivary gland neoplasm as allelic loss structural rearrangement monosomyamp polysomy

IV Other factors Silica dust or wood dust Using Kerosene as cooking fuel Warthinrsquos tumor is strongly associated with cigarette smoking

DiagnosisI Clinical features

Usually present as slowly growing painless swelling A sudden increase in size ( infection cystic degeneration hemorrhage inside the

mass or malignant transformation) malignant indicators asFacial nerve paresis or paralysis Weakness or numbness of

the tongue or in distribution of branches of trigeminal nerve Pain Fixation Cervical adenopathy

History of malignant skin lesion should be clarified as case of metastatic disease to the parotidNeed full examination especially

6

The mass itself Skin of face or scalp Oropharynx C N S Cervical lymph node

II Fine needle aspiration biopsyEfficacy is well established

Accuracy = 84-97 Sensitivity = 54-95 Specificity = 86=100

Safe well tolerated

Opponents

7

Doesnrsquot change management Surgery regardless of reported diagnosis Obscuring final pathologic diagnosis Frequency of ldquoinadequaterdquo sampling requires multiple biopsies prolongs course until definitive treatment increases cost

Proponent Important to distinguish benign vs malignant nature of neoplasm Preoperative patient counseling Surgical planning Differentiate between neoplastic and non-neoplastic processes Avoid surgery in large number of patients

III Radiology Conventional radiography amp sialography are rarely used C T amp M R I give better understanding

o Location amp extent of the tumoro Its relation to major neurovascular structureo Perineural spreado Skull base invasiono Intracranial extensiono But most of the time canrsquot be differentiate between benign amp malignant

entities Nuclear imaging using Technetium 99m pertechnetate is rarely obtained because FNAB may provide better information Other imaging studies Ultrasonography Color doppler sonography

IV Biopsies-Incisional intraoral biopsy of parapharyngeal tumor should not be performed-The proper approach to parotid neoplasm is parotidectomy which diagnostic amp curative- Open biopsy done rarely amp in the patient who not surgical candidate amp for whom FNAB is not diagnostic

Benign tumorsPleomorphic Adenoma

8

Most common of all salivary gland neoplasms 70 of parotid tumors 4th-6th decades FM = 3-41 Slow-growing painlessfirm mass Rarely progresive

1048708 Gross pathology Smooth Well-demarcated Solid Cystic changes Myxoid stroma

1048708 Histology 1048708 Mixture of epithelial myopeithelial and stromal components 1048708 No true capsule 1048708 Tumor pseudopods

Warthinrsquos Tumor papillary cystadenoma lymphomatosum 6-10 of parotid neoplasms Older Caucasian males 10 bilateral or multicentric 3 with associated neoplasms Presentation slow-growing painless mass

Gross pathology Encapsulated Smoothlobulated surface Cystic spaces of variable size with viscous fluid shaggy epithelium Solid areas with white nodules representing lymphoid follicles

Histology1048708Papillary projections into cystic spaces surrounded by lymphoid stroma

Oncocytoma lt 1of salivary gand tumors Parotid is the most frequent site Slow-growing painless mass 6th decade

9

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

4

PathophysiologyI Multi-cellular theory Salivary gland arise from the adult differentiated counter part of salivary glandunit10487081048708 Acinar cell acinous tumor10487081048708 Striated duct cell oncocytic tumor10487081048708 Intercalated duct cell mixed tumor10487081048708 Excretory duct cells mucoepidermoid carcinoma amp squamous cell carcinoma

5

II Bicellular Theory The basal cell of excretory amp intercalated duct act as stem cells10487081048708 Intercalated duct stem cell acinous cell carcinoma adenoid cystic carcinoma mixed tumor amp oncocytic tumors10487081048708 Excretory duct stem cell squamous cell carcinoma amp mucoepidermoid carcinoma

AetiologyI Radiation Several studies have implicate radiation associated neoplasm Does-response pattern Mostly parotid Commonly mucoepidermoid carcinoma

II Epstein-Barr Virus The consistent association of E B V with lymphoepithelial carcinoma of the

salivary gland suggest the virus probably plays causal role Similar pathogenesis of nasopharyngeal carcinoma no evidence of a causal role of EBV in other primary tumors of the salivary gland

III Genetic FactorsMany genetic alternation may be responsible for increased likelihood of developing salivary gland neoplasm as allelic loss structural rearrangement monosomyamp polysomy

IV Other factors Silica dust or wood dust Using Kerosene as cooking fuel Warthinrsquos tumor is strongly associated with cigarette smoking

DiagnosisI Clinical features

Usually present as slowly growing painless swelling A sudden increase in size ( infection cystic degeneration hemorrhage inside the

mass or malignant transformation) malignant indicators asFacial nerve paresis or paralysis Weakness or numbness of

the tongue or in distribution of branches of trigeminal nerve Pain Fixation Cervical adenopathy

History of malignant skin lesion should be clarified as case of metastatic disease to the parotidNeed full examination especially

6

The mass itself Skin of face or scalp Oropharynx C N S Cervical lymph node

II Fine needle aspiration biopsyEfficacy is well established

Accuracy = 84-97 Sensitivity = 54-95 Specificity = 86=100

Safe well tolerated

Opponents

7

Doesnrsquot change management Surgery regardless of reported diagnosis Obscuring final pathologic diagnosis Frequency of ldquoinadequaterdquo sampling requires multiple biopsies prolongs course until definitive treatment increases cost

Proponent Important to distinguish benign vs malignant nature of neoplasm Preoperative patient counseling Surgical planning Differentiate between neoplastic and non-neoplastic processes Avoid surgery in large number of patients

III Radiology Conventional radiography amp sialography are rarely used C T amp M R I give better understanding

o Location amp extent of the tumoro Its relation to major neurovascular structureo Perineural spreado Skull base invasiono Intracranial extensiono But most of the time canrsquot be differentiate between benign amp malignant

entities Nuclear imaging using Technetium 99m pertechnetate is rarely obtained because FNAB may provide better information Other imaging studies Ultrasonography Color doppler sonography

IV Biopsies-Incisional intraoral biopsy of parapharyngeal tumor should not be performed-The proper approach to parotid neoplasm is parotidectomy which diagnostic amp curative- Open biopsy done rarely amp in the patient who not surgical candidate amp for whom FNAB is not diagnostic

Benign tumorsPleomorphic Adenoma

8

Most common of all salivary gland neoplasms 70 of parotid tumors 4th-6th decades FM = 3-41 Slow-growing painlessfirm mass Rarely progresive

1048708 Gross pathology Smooth Well-demarcated Solid Cystic changes Myxoid stroma

1048708 Histology 1048708 Mixture of epithelial myopeithelial and stromal components 1048708 No true capsule 1048708 Tumor pseudopods

Warthinrsquos Tumor papillary cystadenoma lymphomatosum 6-10 of parotid neoplasms Older Caucasian males 10 bilateral or multicentric 3 with associated neoplasms Presentation slow-growing painless mass

Gross pathology Encapsulated Smoothlobulated surface Cystic spaces of variable size with viscous fluid shaggy epithelium Solid areas with white nodules representing lymphoid follicles

Histology1048708Papillary projections into cystic spaces surrounded by lymphoid stroma

Oncocytoma lt 1of salivary gand tumors Parotid is the most frequent site Slow-growing painless mass 6th decade

9

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

PathophysiologyI Multi-cellular theory Salivary gland arise from the adult differentiated counter part of salivary glandunit10487081048708 Acinar cell acinous tumor10487081048708 Striated duct cell oncocytic tumor10487081048708 Intercalated duct cell mixed tumor10487081048708 Excretory duct cells mucoepidermoid carcinoma amp squamous cell carcinoma

5

II Bicellular Theory The basal cell of excretory amp intercalated duct act as stem cells10487081048708 Intercalated duct stem cell acinous cell carcinoma adenoid cystic carcinoma mixed tumor amp oncocytic tumors10487081048708 Excretory duct stem cell squamous cell carcinoma amp mucoepidermoid carcinoma

AetiologyI Radiation Several studies have implicate radiation associated neoplasm Does-response pattern Mostly parotid Commonly mucoepidermoid carcinoma

II Epstein-Barr Virus The consistent association of E B V with lymphoepithelial carcinoma of the

salivary gland suggest the virus probably plays causal role Similar pathogenesis of nasopharyngeal carcinoma no evidence of a causal role of EBV in other primary tumors of the salivary gland

III Genetic FactorsMany genetic alternation may be responsible for increased likelihood of developing salivary gland neoplasm as allelic loss structural rearrangement monosomyamp polysomy

IV Other factors Silica dust or wood dust Using Kerosene as cooking fuel Warthinrsquos tumor is strongly associated with cigarette smoking

DiagnosisI Clinical features

Usually present as slowly growing painless swelling A sudden increase in size ( infection cystic degeneration hemorrhage inside the

mass or malignant transformation) malignant indicators asFacial nerve paresis or paralysis Weakness or numbness of

the tongue or in distribution of branches of trigeminal nerve Pain Fixation Cervical adenopathy

History of malignant skin lesion should be clarified as case of metastatic disease to the parotidNeed full examination especially

6

The mass itself Skin of face or scalp Oropharynx C N S Cervical lymph node

II Fine needle aspiration biopsyEfficacy is well established

Accuracy = 84-97 Sensitivity = 54-95 Specificity = 86=100

Safe well tolerated

Opponents

7

Doesnrsquot change management Surgery regardless of reported diagnosis Obscuring final pathologic diagnosis Frequency of ldquoinadequaterdquo sampling requires multiple biopsies prolongs course until definitive treatment increases cost

Proponent Important to distinguish benign vs malignant nature of neoplasm Preoperative patient counseling Surgical planning Differentiate between neoplastic and non-neoplastic processes Avoid surgery in large number of patients

III Radiology Conventional radiography amp sialography are rarely used C T amp M R I give better understanding

o Location amp extent of the tumoro Its relation to major neurovascular structureo Perineural spreado Skull base invasiono Intracranial extensiono But most of the time canrsquot be differentiate between benign amp malignant

entities Nuclear imaging using Technetium 99m pertechnetate is rarely obtained because FNAB may provide better information Other imaging studies Ultrasonography Color doppler sonography

IV Biopsies-Incisional intraoral biopsy of parapharyngeal tumor should not be performed-The proper approach to parotid neoplasm is parotidectomy which diagnostic amp curative- Open biopsy done rarely amp in the patient who not surgical candidate amp for whom FNAB is not diagnostic

Benign tumorsPleomorphic Adenoma

8

Most common of all salivary gland neoplasms 70 of parotid tumors 4th-6th decades FM = 3-41 Slow-growing painlessfirm mass Rarely progresive

1048708 Gross pathology Smooth Well-demarcated Solid Cystic changes Myxoid stroma

1048708 Histology 1048708 Mixture of epithelial myopeithelial and stromal components 1048708 No true capsule 1048708 Tumor pseudopods

Warthinrsquos Tumor papillary cystadenoma lymphomatosum 6-10 of parotid neoplasms Older Caucasian males 10 bilateral or multicentric 3 with associated neoplasms Presentation slow-growing painless mass

Gross pathology Encapsulated Smoothlobulated surface Cystic spaces of variable size with viscous fluid shaggy epithelium Solid areas with white nodules representing lymphoid follicles

Histology1048708Papillary projections into cystic spaces surrounded by lymphoid stroma

Oncocytoma lt 1of salivary gand tumors Parotid is the most frequent site Slow-growing painless mass 6th decade

9

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

II Bicellular Theory The basal cell of excretory amp intercalated duct act as stem cells10487081048708 Intercalated duct stem cell acinous cell carcinoma adenoid cystic carcinoma mixed tumor amp oncocytic tumors10487081048708 Excretory duct stem cell squamous cell carcinoma amp mucoepidermoid carcinoma

AetiologyI Radiation Several studies have implicate radiation associated neoplasm Does-response pattern Mostly parotid Commonly mucoepidermoid carcinoma

II Epstein-Barr Virus The consistent association of E B V with lymphoepithelial carcinoma of the

salivary gland suggest the virus probably plays causal role Similar pathogenesis of nasopharyngeal carcinoma no evidence of a causal role of EBV in other primary tumors of the salivary gland

III Genetic FactorsMany genetic alternation may be responsible for increased likelihood of developing salivary gland neoplasm as allelic loss structural rearrangement monosomyamp polysomy

IV Other factors Silica dust or wood dust Using Kerosene as cooking fuel Warthinrsquos tumor is strongly associated with cigarette smoking

DiagnosisI Clinical features

Usually present as slowly growing painless swelling A sudden increase in size ( infection cystic degeneration hemorrhage inside the

mass or malignant transformation) malignant indicators asFacial nerve paresis or paralysis Weakness or numbness of

the tongue or in distribution of branches of trigeminal nerve Pain Fixation Cervical adenopathy

History of malignant skin lesion should be clarified as case of metastatic disease to the parotidNeed full examination especially

6

The mass itself Skin of face or scalp Oropharynx C N S Cervical lymph node

II Fine needle aspiration biopsyEfficacy is well established

Accuracy = 84-97 Sensitivity = 54-95 Specificity = 86=100

Safe well tolerated

Opponents

7

Doesnrsquot change management Surgery regardless of reported diagnosis Obscuring final pathologic diagnosis Frequency of ldquoinadequaterdquo sampling requires multiple biopsies prolongs course until definitive treatment increases cost

Proponent Important to distinguish benign vs malignant nature of neoplasm Preoperative patient counseling Surgical planning Differentiate between neoplastic and non-neoplastic processes Avoid surgery in large number of patients

III Radiology Conventional radiography amp sialography are rarely used C T amp M R I give better understanding

o Location amp extent of the tumoro Its relation to major neurovascular structureo Perineural spreado Skull base invasiono Intracranial extensiono But most of the time canrsquot be differentiate between benign amp malignant

entities Nuclear imaging using Technetium 99m pertechnetate is rarely obtained because FNAB may provide better information Other imaging studies Ultrasonography Color doppler sonography

IV Biopsies-Incisional intraoral biopsy of parapharyngeal tumor should not be performed-The proper approach to parotid neoplasm is parotidectomy which diagnostic amp curative- Open biopsy done rarely amp in the patient who not surgical candidate amp for whom FNAB is not diagnostic

Benign tumorsPleomorphic Adenoma

8

Most common of all salivary gland neoplasms 70 of parotid tumors 4th-6th decades FM = 3-41 Slow-growing painlessfirm mass Rarely progresive

1048708 Gross pathology Smooth Well-demarcated Solid Cystic changes Myxoid stroma

1048708 Histology 1048708 Mixture of epithelial myopeithelial and stromal components 1048708 No true capsule 1048708 Tumor pseudopods

Warthinrsquos Tumor papillary cystadenoma lymphomatosum 6-10 of parotid neoplasms Older Caucasian males 10 bilateral or multicentric 3 with associated neoplasms Presentation slow-growing painless mass

Gross pathology Encapsulated Smoothlobulated surface Cystic spaces of variable size with viscous fluid shaggy epithelium Solid areas with white nodules representing lymphoid follicles

Histology1048708Papillary projections into cystic spaces surrounded by lymphoid stroma

Oncocytoma lt 1of salivary gand tumors Parotid is the most frequent site Slow-growing painless mass 6th decade

9

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

The mass itself Skin of face or scalp Oropharynx C N S Cervical lymph node

II Fine needle aspiration biopsyEfficacy is well established

Accuracy = 84-97 Sensitivity = 54-95 Specificity = 86=100

Safe well tolerated

Opponents

7

Doesnrsquot change management Surgery regardless of reported diagnosis Obscuring final pathologic diagnosis Frequency of ldquoinadequaterdquo sampling requires multiple biopsies prolongs course until definitive treatment increases cost

Proponent Important to distinguish benign vs malignant nature of neoplasm Preoperative patient counseling Surgical planning Differentiate between neoplastic and non-neoplastic processes Avoid surgery in large number of patients

III Radiology Conventional radiography amp sialography are rarely used C T amp M R I give better understanding

o Location amp extent of the tumoro Its relation to major neurovascular structureo Perineural spreado Skull base invasiono Intracranial extensiono But most of the time canrsquot be differentiate between benign amp malignant

entities Nuclear imaging using Technetium 99m pertechnetate is rarely obtained because FNAB may provide better information Other imaging studies Ultrasonography Color doppler sonography

IV Biopsies-Incisional intraoral biopsy of parapharyngeal tumor should not be performed-The proper approach to parotid neoplasm is parotidectomy which diagnostic amp curative- Open biopsy done rarely amp in the patient who not surgical candidate amp for whom FNAB is not diagnostic

Benign tumorsPleomorphic Adenoma

8

Most common of all salivary gland neoplasms 70 of parotid tumors 4th-6th decades FM = 3-41 Slow-growing painlessfirm mass Rarely progresive

1048708 Gross pathology Smooth Well-demarcated Solid Cystic changes Myxoid stroma

1048708 Histology 1048708 Mixture of epithelial myopeithelial and stromal components 1048708 No true capsule 1048708 Tumor pseudopods

Warthinrsquos Tumor papillary cystadenoma lymphomatosum 6-10 of parotid neoplasms Older Caucasian males 10 bilateral or multicentric 3 with associated neoplasms Presentation slow-growing painless mass

Gross pathology Encapsulated Smoothlobulated surface Cystic spaces of variable size with viscous fluid shaggy epithelium Solid areas with white nodules representing lymphoid follicles

Histology1048708Papillary projections into cystic spaces surrounded by lymphoid stroma

Oncocytoma lt 1of salivary gand tumors Parotid is the most frequent site Slow-growing painless mass 6th decade

9

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

Doesnrsquot change management Surgery regardless of reported diagnosis Obscuring final pathologic diagnosis Frequency of ldquoinadequaterdquo sampling requires multiple biopsies prolongs course until definitive treatment increases cost

Proponent Important to distinguish benign vs malignant nature of neoplasm Preoperative patient counseling Surgical planning Differentiate between neoplastic and non-neoplastic processes Avoid surgery in large number of patients

III Radiology Conventional radiography amp sialography are rarely used C T amp M R I give better understanding

o Location amp extent of the tumoro Its relation to major neurovascular structureo Perineural spreado Skull base invasiono Intracranial extensiono But most of the time canrsquot be differentiate between benign amp malignant

entities Nuclear imaging using Technetium 99m pertechnetate is rarely obtained because FNAB may provide better information Other imaging studies Ultrasonography Color doppler sonography

IV Biopsies-Incisional intraoral biopsy of parapharyngeal tumor should not be performed-The proper approach to parotid neoplasm is parotidectomy which diagnostic amp curative- Open biopsy done rarely amp in the patient who not surgical candidate amp for whom FNAB is not diagnostic

Benign tumorsPleomorphic Adenoma

8

Most common of all salivary gland neoplasms 70 of parotid tumors 4th-6th decades FM = 3-41 Slow-growing painlessfirm mass Rarely progresive

1048708 Gross pathology Smooth Well-demarcated Solid Cystic changes Myxoid stroma

1048708 Histology 1048708 Mixture of epithelial myopeithelial and stromal components 1048708 No true capsule 1048708 Tumor pseudopods

Warthinrsquos Tumor papillary cystadenoma lymphomatosum 6-10 of parotid neoplasms Older Caucasian males 10 bilateral or multicentric 3 with associated neoplasms Presentation slow-growing painless mass

Gross pathology Encapsulated Smoothlobulated surface Cystic spaces of variable size with viscous fluid shaggy epithelium Solid areas with white nodules representing lymphoid follicles

Histology1048708Papillary projections into cystic spaces surrounded by lymphoid stroma

Oncocytoma lt 1of salivary gand tumors Parotid is the most frequent site Slow-growing painless mass 6th decade

9

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

Most common of all salivary gland neoplasms 70 of parotid tumors 4th-6th decades FM = 3-41 Slow-growing painlessfirm mass Rarely progresive

1048708 Gross pathology Smooth Well-demarcated Solid Cystic changes Myxoid stroma

1048708 Histology 1048708 Mixture of epithelial myopeithelial and stromal components 1048708 No true capsule 1048708 Tumor pseudopods

Warthinrsquos Tumor papillary cystadenoma lymphomatosum 6-10 of parotid neoplasms Older Caucasian males 10 bilateral or multicentric 3 with associated neoplasms Presentation slow-growing painless mass

Gross pathology Encapsulated Smoothlobulated surface Cystic spaces of variable size with viscous fluid shaggy epithelium Solid areas with white nodules representing lymphoid follicles

Histology1048708Papillary projections into cystic spaces surrounded by lymphoid stroma

Oncocytoma lt 1of salivary gand tumors Parotid is the most frequent site Slow-growing painless mass 6th decade

9

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

MF = 11Gross1048708 Encapsulated1048708Homogeneous smooth1048708Orangerust colorHistology

Cords of uniform cells and thin fibrous stroma Large polyhedral cells Distinct cell membrane Granular eosinophilic cytoplasm Central round vesicular Nucleus

Monomorphic Adenomas lt 5 of salivary gland tumors Only one morphologic cell type Subclassified into

bull Basal Cell Adenomabull Canalicular Adenomabull Myoepitheliomabull Clear cell adenomabull glycogen-rich adenomabull Sebaceous adenoma

Basal Cell Adenoma Basal cell is the most common 18 of benign epithelial salivary gland neoplasms 6th decade MF = approximately 11 Caucasian gt African American Most common in parotid Histological types Solid Trabecular Tubular Membranous

Hemangioma Benign of endothelial origon lt 5 of salivary gland tumors gt 90 of parotid tumors in children lt 1year Present at birth Unilateralpainlessrapid-growth

10

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

F gt M CT amp MRI are dignostic Spontanouus regression surgery if needed

MalignantMucoepidermoid Carcinoma

Most common salivary gland malignancy 5-9 of salivary neoplasms Parotid 80 - 90 of cases 3rd-8th decades peak in 5th decade FgtM Caucasian gt African American Most common salivary gland tumor in children It contain two major elements mucin producing cells amp epithelial cells of

epidermoid variety amp according to that classified into low-grade high grade amp intermediate

Presentation10487081048708 Low-grade slow growing painless mass10487081048708 High-grade rapidly enlarging +- painGross pathology-Well-circumscribed to partially encapsulated to unencapsulated-Solid tumor with cystic spacesHistologymdashLow-grade

1048708Mucus cell gt epidermoid cells 1048708 Prominent cysts 1048708 Mature cellular elements

HistologymdashIntermediategrade 1048708 Mucus = epidermoid 1048708 Fewer and smaller cysts 1048708 Increasing pleomorphism and mitotic figures

Histologymdash High-grade Epidermoid gt mucus Solid tumor cell proliferation Mistaken for SCCA-Mucin staining high chance of locoregional recurrencedistant metastasis amp shorter survival rate

compaired with low grade tumors

Adenoid Cystic Carcinoma Overall 2nd most common malignancy

11

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

Most common in submandibular sublingual and minor salivary glands M = F 5th decade Presentation

10487081048708 Asymptomatic enlarging mass10487081048708 Pain paresthesias facial weaknessparalysis

Subclassified histologically inCribriform tubular solid Perineural involvement is characteristic of adenoid cystic carcinoma amp occur in

20-80 Lymphatic spread uncommon 15 Distant metastasis continue to occur for as long as 20 years or more amp common in

lung (90)

Acinic Cell Carcinoma 3 of all salivary gland neoplasm 80-90 in parotid 15 of all parotid tumors Main age is 40-50 years 23 Occur in female 3 Shows bilateral involvement Second most common salivary gland in children Well circumscribed tumor with fibrosis tissue macroscopically Four histological patterns identified solid microcystic papillary cysticamp follicular

But the type of pattern do not correlate with biologic behavior or prognosis It is generally regarded as relatively low grade malignancy amp has more favorable

prognosis They seldom metastasize but they have high tendency to recur locally

Malignant mixed tumor Epithelial amp mesenchymal elements 3-12 of salivary gland tumor frac34 in parotid Carcinoma ex-pleomorphic adenoma (most common variety) Incidence increase by duration amp recurrence of pleomorphic adenoma Microscopic foci of necrosis hemorrhage calcification or excessive hyalinization 2 Subgroups invasive amp non-invasive Degree of the invasion determine the prognosis which lt8-mm 5-year survival rate

is 100but gt 8- mm show lt50

12

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

Invasive type show 25 regional amp 33 distant metastasis

Polymorphous low grade adenocarcinoma Known also as terminal duct or lobular carcinoma Majority in oral cavity (palate or buccal mucosa) Slow growth Common in female 21 Lymph node metastasis 9 Local recurrence 17 No distant metastasis

Salivary duct carcinoma High grade aggressive tumor from excretory duct cells in major salivary gland

mainly Microscopic feature remarkably similar to mammary intraductal carcinoma 35 recurrence 62 distant metastasis 77 died of the disease

Epithelial-Myoepithelial carcinoma 1 of salivary gland neoplasm Ductal cell population Mostly involve the parotid at age 50-80 female Sold tubular amp cribriform subtypes High incidence of local recurrence 50 especially with sold type Distant metastasis 25 40 of patient died by their disease

Adenocarcinoma Ranging from low grade well differentiated to high grade invasive lesions Common in major gland Originate from excretory or striated ducts Cystic or solid may be papillary or non-papillary in growth pattern Prognosis depend on grade

Squamous cell carcinoma Rare to be primary amp common to be secondary from intraparotid lymph node or

direct involvement Should be differentiated from mucoepidermoid carcinoma by presence of mucin on

electron microscope with stain or immunohistochemical test Incidence 03 -15 in major gland

13

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

50 nodal metastasis MF = 21 7th-8th decades TRsurgery + Neck dissection + PostopXRT

Undifferentiated Carcinoma Lack of histological characteristics High in china Indistinguishable histological from undifferentiated carcinoma of nasopharynx Linked to E B virus

Lymphoma 5 of all extranodal lymphoma affected salivary gland 90 occur in parotid 85 are N H L Nodal or extranodal in the salivary gland Primary or disseminated Its risk in Sjogrenrsquos syndrome is 44 fold higher amp has bad prognosis

Secondary tumorsThe majority of metastasis are caused by

lymphatic spread from cutanous malignancy of the head amp neck Hematogenous metastasis are rare amp majority from lung kidney amp breast Contiguous extension of facial sarcomas

10 of malignant parotid gland tumors(2ry) 40 are SCC amp 40 are melanoma 23 of SCCs occur in 1st year Melanoma correlate with thickness of primary tumor

ManagementStaging

14

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

Treatment for salivary gland cancerThe principal treatment of salivary gland tumors is surgical resection with save margins used either as single modality or conjunction with adjuvant radiotherapy

I Surgery Small localized tumor of the superficial lobe may be adequately treated with

superficial parotidectomy Large tumor and tumor involving the deep lobe usually require total

parotidectomy The facial nerve is dissected amp preserved unless it is directly involved by the

tumor 1048708 1048708 Preoperative weakness or paralysis 1048708 1048708 Intraoperative evidence of gross or microscopic infiltration The sacrificing more likely with large amp high grade tumor or that one extend from

superficial to deep lobe Surgical margin on distal amp proximal nerve stumps should be checked due to the

possibility of perineural spread Tumors extending beyond the parotid gland may require resection of surrounding

structures up to subtotal petrosectomy

15

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

Complication of Parotidectomy1) Freyrsquos syndrome (Gustatory sweating)

Symptoms vary in severity Incidence 35-60 Due to cross re-innervation between the postganglionic secretomotor

parasympathetic fibers to the parotid gland amp the postganglionic sympathetic fibers supplying the sweat glands of the skin

Diagnosis depend largely on the patientrsquos symptoms but can be confirm by Minorrsquos starch amp iodine test

If the symptoms are bothersome we can use antiperspirant glycopyrrolate lotion tympanic neurectomy or muscle flap or sheet of facia between the skin and the parotid ped

2) Facial nerve paresis or paralysis3) Salivary fistula

1048708 Uncommon amp self limited 1048708 Clear sialorrhea or fluid collections 1048708 Treated by wound care pressure dressing repeated aspiration of fluid

collections or oral anticholinergics

II Neck Dissection If there is metastatic cervical LAP the patient need comprehensive cervical

lymphadenectomy But there is controversy about management of clinically negative neck In high-grade or large tumor The incidence of occult regional disease is relatively

high so the elective neck dissection or selective (supraomohyoid) neck dissection should be considered

In low-grade malignancy the elective neck dissection not recommended

IIIRadiotherapy Adjuvant radiotherapy is superior to surgery alone amp effective in improve

locoregional control amp highly recommended in patient with poor prognosis In inoperable tumor the neutron irradiation alone is the therapy of choice amp

superior to conventional proton or electron therapy amp even superior to debulking surgical procedure

IV Chemotherapy

16

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

Although the salivary gland malignancy show some response to various chemotherapeutic agents these response have not resulted in significant improvement in long survival rate

PrognosisI- StageThe most important factor The incidence of local recurrence amp regional metastasis are lowest in patient with stageI

II-Histology amp Grade The biologic behavior depend largely on the histologic type of malignancy Squamous cell carcinoma malignant mixed tumors undifferentiated carcinoma amp

salivary duct carcinoma are considered high-grade tumors while acinic cell carcinoma amp polymorphous low grade adenocarcinoma are considered low-grade tumor

Adenoid cystic carcinoma considered a high grade malignancy although histologic pattern have different biologic behavior

Mucoepidermoid carcinoma highly correlated with tumor grade

III Site The prognosis amp also the local recurrence after treatment have a definite correlations with primary site of origin which better in major salivary glands than minor salivary glands mainly due to- Present at more advance stage- High incidence of extension amp fixation- Bone involvement

IV Nodal metastasis Considered as predictor of poor prognosis Adenoid cyst carcinoma 10amp20 years survival rate drop from 62 amp 50 to 38 amp

8 with nodal metastasis

V Surgical margins Some consider it as the most important factor Microscopic positive margin need radiotherapy to achieve good prognosis

VI Perineural spread In squamous cell carcinoma the perineural spread show poor prognosis The effect of perineural spread in the prognosis of adenoid cystic carcinoma still

controversial But any how the perineural spread in major nerve indicate adverse prognostic factor

VII Facial Nerve paralysis

17

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18

Although facial nerve paralysis may not be associated with 100 mortality rate it is an indicator of poor prognosis

VIII Pain Patients with pain appear to have a less favorable outcome Its presence increase likelihood of local invasion of bone or sensory nerves

IX Distant metastasis 20 of parotid malignancy Most frequently in adenoid cystic carcinoma amp undifferentiated carcinoma Lung bone amp brain

X GenderMen have poorer outcome

18