Lymphomas of the Lymphomas of the Head and NeckHead and Neck

Tal Marom, MDTal Marom, MD

LymphomaLymphoma

Cancer of the lymphatic systemCancer of the lymphatic system Lymphoma is differentiated by the type of Lymphoma is differentiated by the type of

cell that multiplies and how the cancer cell that multiplies and how the cancer presents itselfpresents itself

Two main groups: Hodkgin’s disease and Two main groups: Hodkgin’s disease and NHLNHL

US prevalence (HD) = 3 cases/100,000 US prevalence (HD) = 3 cases/100,000 (incidence ↓)(incidence ↓)

US prevalence (NHL)=16 cases/100,000 US prevalence (NHL)=16 cases/100,000 (incidence↑)(incidence↑)

Hodgkin vs. Non-Hodgkin LyHodgkin vs. Non-Hodgkin Ly..Hodgkin's Lymphoma

Non-Hodgkin's Lymphomas

AgeAverage age is 27.7 with two age peaks, the major one between 15 and 24 with a lesser peak after age 55.

Average age is about 67. 

Lifetime prevalence

Men 0.23%Women 0.20%

Men 2.12%Women 1.79%

OccurrenceAbout 15% of all lymphomas

About 85% of all lymphomas

Hodgkin vs. Non-Hodgkin LyHodgkin vs. Non-Hodgkin Ly..Hodgkin's 

LymphomaNon-Hodgkin's Lymphomas

LocationLocation LN above the collar bone. LN above the collar bone. In Hodgkin's it is also In Hodgkin's it is also more likely to appear more likely to appear in the chest cavity in the chest cavity (mediastinum), (mediastinum), particularly in younger particularly in younger patients.patients.Only about 15% to Only about 15% to 20% of cases are 20% of cases are below the diaphragm.below the diaphragm.

Extra-nodular disease in Extra-nodular disease in about 4% of cases.about 4% of cases.

LN above the collar bone. In LN above the collar bone. In NHL it is also more likely NHL it is also more likely

to appear in the to appear in the mesenteric nodes in the mesenteric nodes in the

abdomen.abdomen.

The disease occurs in the The disease occurs in the chest cavity in less than chest cavity in less than

40% of patients. (An 40% of patients. (An exception, lymphoblastic exception, lymphoblastic lymphoma, which is seen lymphoma, which is seen

most often in young most often in young people, is likely to first people, is likely to first appear in the chest.)appear in the chest.)

Extra-nodular disease in Extra-nodular disease in about 23% of patients. about 23% of patients.

Slow-growing lymphomas Slow-growing lymphomas are common in the liver are common in the liver

and bone marrow.and bone marrow.

Hodgkin vs. Non-Hodgkin LyHodgkin vs. Non-Hodgkin Ly..Hodgkin's Lymphoma

Non-Hodgkin's Lymphomas

Affected Lymph Cells

B-Lymphocytescharacterized by the Reed-

Sternberg Cell

B-Lymphocytes (>90%), T-Lymphocytes or Natural

Killer (NK) Cells

Symptoms More likely (40%) to have systemic ("B") symptoms at the

time of diagnosis.

Less likely than HL to have systemic ("B") symptoms

(27%) at the time of diagnosis.

ProgressionLess likely to be diagnosed in stage IV (10%).

Hodgkin's disease usually progresses slowly (or

aggressively) in an orderly way from one lymph node region to the

next. If it spreads below the diaphragm, it usually reaches the spleen first; the disease then may spread to the liver and bone marrow. If the disease starts in the nodes in the middle of the chest, it may spread

outward to the chest wall and areas around the heart and lungs.

More likely than HD to be diagnosed in stage IV (36%)

but this will vary by NHL subtype. 

The Non-Hodgkin's lymphomas are less

predictable in their course than Hodgkin's and they are

more apt to spread

Reed Sternberg Cell Reed Sternberg Cell

H&N LymphomaH&N Lymphoma

Lymphoma is the second most common primary Lymphoma is the second most common primary malignancy occurring in the head and neck, and malignancy occurring in the head and neck, and incidence of aggressive non-Hodgkin lymphoma is incidence of aggressive non-Hodgkin lymphoma is

rising in young and middle-aged patients.rising in young and middle-aged patients. 25% of all extra-nodal lymphomas occur in the head 25% of all extra-nodal lymphomas occur in the head

and neck, and 8% of supraclavicular fine-needle and neck, and 8% of supraclavicular fine-needle aspirates are diagnosed as lymphoma.aspirates are diagnosed as lymphoma.

REALREAL classification classification (Revised European American (Revised European American Lymphoid neoplasm) : indolent, aggressive and Lymphoid neoplasm) : indolent, aggressive and Hodgkin diseaseHodgkin disease

Clinical presentationClinical presentation

Neck- lymphadenopathy, ulcerated massNeck- lymphadenopathy, ulcerated mass Oropharynx- enlarged tonsil, tongue base Oropharynx- enlarged tonsil, tongue base

thickeningthickening Nasopharynx – mass, SOMNasopharynx – mass, SOM Nose & paranasal sinuses- ulcerated destructive Nose & paranasal sinuses- ulcerated destructive

lesion –susp. NK/T cell Lymphoma (“lesion –susp. NK/T cell Lymphoma (“midline lethal midline lethal granuloma”granuloma”), associated with EBV), associated with EBV

Thyroid - neck swelling, hoarseness, dysphagia, or Thyroid - neck swelling, hoarseness, dysphagia, or neck pressure/tenderness neck pressure/tenderness

Salivary glands- massesSalivary glands- masses Base skull – cranial neuropathy, facial pain, Base skull – cranial neuropathy, facial pain,

hearing loss, vertigo, proptosis, or visual hearing loss, vertigo, proptosis, or visual symptomssymptoms

Physical examinationPhysical examination

History – cough, fever, GI, History – cough, fever, GI, abdominal masses, pruritusabdominal masses, pruritus

Full PE, look for peripheral Full PE, look for peripheral adenopathyadenopathy

Differential diagnosisDifferential diagnosis infectious etiologiesinfectious etiologies

BacteriaBacteriaViruses (eg, infectious mononucleosis, cytomegalovirus, HIV)Viruses (eg, infectious mononucleosis, cytomegalovirus, HIV)Parasites (eg, toxoplasmosis)Parasites (eg, toxoplasmosis)

Nasal granulomatous diseaseNasal granulomatous diseaseWegener granulomatosisWegener granulomatosisLymphomatoid granulomatosisLymphomatoid granulomatosisInfections (eg, leishmaniasis, syphilis, TB)Infections (eg, leishmaniasis, syphilis, TB)

Mediastinal presentationMediastinal presentationInfections (eg, histoplasmosis, tuberculosis)Infections (eg, histoplasmosis, tuberculosis)SarcoidosisSarcoidosisOther neoplasms Other neoplasms

Benign lymphoid hyperplasiasBenign lymphoid hyperplasiasB-cell predominant - Cutaneous lymphoid hyperplasia (CLH), B-cell predominant - Cutaneous lymphoid hyperplasia (CLH), angiolymphoid hyperplasia with eosinophilia, Kimura disease, angiolymphoid hyperplasia with eosinophilia, Kimura disease, and Castleman diseaseand Castleman diseaseT-cell predominant - T-cell CLH, lymphomatoid contact T-cell predominant - T-cell CLH, lymphomatoid contact dermatitis, and lymphomatoid drug eruptiondermatitis, and lymphomatoid drug eruption

Other neoplasmsOther neoplasmsSquamous cell carcinomaSquamous cell carcinomaNasopharyngeal carcinomaNasopharyngeal carcinomaThyroid carcinoma Thyroid carcinoma

Lab studiesLab studies

CBCCBC Chemistry, liver, LDHChemistry, liver, LDH ESRESR UrineUrine Serum Serum ββ2 2 microglobulin (worse prognosis)microglobulin (worse prognosis) CXRCXR Total body CTTotal body CT Galium scanningGalium scanning Genetic studiesGenetic studies

ProceduresProcedures

Fiberoscopy- NPH, larynxFiberoscopy- NPH, larynx FNA-C from neck massFNA-C from neck mass Excisional/Incisional BxExcisional/Incisional Bx Diagnostic TEDiagnostic TE (Bone marrow, LP, liver, (Bone marrow, LP, liver,

explorative laparotomy…)explorative laparotomy…)

Ann Arbor Lymphoma stagingAnn Arbor Lymphoma staging Stage I - Involvement of a single LN region/ Stage I - Involvement of a single LN region/

lymphoid structure lymphoid structure Stage II - Involvement of 2 or more LN regions Stage II - Involvement of 2 or more LN regions

on the same side of the diaphragm or on the same side of the diaphragm or localized contiguous involvement of localized contiguous involvement of only one extra-lymphatic site and LN only one extra-lymphatic site and LN region region

Stage III - Involvement of LN regions or Stage III - Involvement of LN regions or lymphoid structures on both sides of lymphoid structures on both sides of the diaphragm the diaphragm

Stage IV - Disseminated involvement of one or Stage IV - Disseminated involvement of one or more extra- lymphatic organs with or more extra- lymphatic organs with or

without LN involvement and/or without LN involvement and/or involvement of the bone marrow or involvement of the bone marrow or

liver liver

TreatmentTreatment

Chemotherapy – major Chemotherapy – major modalitymodality

RadiotherapyRadiotherapy Surgery ???Surgery ??? Relapse !!!Relapse !!!

ChemotherapyChemotherapy

Hodgkin disease – Hodgkin disease – - MOPP- MOPP (mechlorethamine [nitrogen mustard], (mechlorethamine [nitrogen mustard],

vincristine, procarbazine, and prednisolone)vincristine, procarbazine, and prednisolone)

[2% post-Rx ALL, infertility][2% post-Rx ALL, infertility]- - ABVDABVD ((doxorubicin (Adriamycin), bleomycin, doxorubicin (Adriamycin), bleomycin,

vinblastine, and dacarbazine)vinblastine, and dacarbazine) - For advanced disease : - For advanced disease :

BEACOPPBEACOPP (cyclophosphamide, doxorubicin, (cyclophosphamide, doxorubicin, etoposide, procarbazine, prednisolone, vincristine, and etoposide, procarbazine, prednisolone, vincristine, and bleomycin with granulocyte colony-stimulating factor) bleomycin with granulocyte colony-stimulating factor)

ChemotherapyChemotherapy

Non-Hodgkin Lymphoma: Non-Hodgkin Lymphoma: CHOP CHOP (cyclophosphamide, doxorubicin, vincristine, and

prednisolone)

Purine analogues

Other proceduresOther procedures

Stem cell transplantationStem cell transplantation IL-2IL-2 Recomninat INF-Recomninat INF-αα Bone marrow transplantation?Bone marrow transplantation?

RadiotherapyRadiotherapy

Mantle fieldMantle field includes the includes the submandibular, cervical, submandibular, cervical, supraclavicular, infraclavicular, supraclavicular, infraclavicular, axillary, mediastinal, and hilar lymph axillary, mediastinal, and hilar lymph nodes nodes

It can be extended to cover the It can be extended to cover the Waldeyer ring and the skull base Waldeyer ring and the skull base with lateral portals for lymphomas with lateral portals for lymphomas involving the skull base involving the skull base

Combined= Chemo + RadioCombined= Chemo + Radio

Surgical treatmentSurgical treatment

Excision of necrotic tissue may be Excision of necrotic tissue may be necessary in nasal NK/T-cell necessary in nasal NK/T-cell lymphoma lymphoma

MALT lymphomas are often treated MALT lymphomas are often treated surgically with or without local surgically with or without local radiotherapy radiotherapy

Lymphomas of the CNS and skull Lymphomas of the CNS and skull base are treated with surgical base are treated with surgical decompression when necessarydecompression when necessary