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Pediatric Neck Masses

Mark Domanski, M.D. Michael Underbrink, M.D. Dept. of Otolaryngology University of Texas Medical Branch, Galveston October 31st, 2007

1

Total % of total

Congeital lesions 244 55% Branchial cleft cyst 78 18% Thyroglossal duct cyst 73 16% Dermoid cyst 43 10% Lymphangioma 34 8% Hemangioma 10 2% Teratoma 2 Bronchogenic cyst 2 Thymic cyst 1 Myelomeningocele 1 Inflammatory lesions 118 27% Reactive lympadenopathy 71 16% Undetermined etiology 66 15% Sinus histiocytosis 5 1% Granulomatous disease 32 7% Atypical mycobacteria 20 4% Cat scratch disease 6 1% Toxoplasmosis 2 Sarcoid 2 Suppurative lympadenitis 10 2% Sialadenitis 5 1%

Non-infammatory benign lesions

23 5%

Inclusion cyst 13 3% Fibromatosis 9 2% Keliod 1 Benign neoplasms 12 3% Neurofibroma 3 1% Lipoma 3 1% Lipoblastoma 2 Paraganglioma 1 Goiter 1 Benign mixed tumor 1 Osteoblastoma 1 Malignant neoplasms 48 11% Lymphoma 34 8% Hodgkin's 23 5% Non-Hodkin's 11 2% Thyroid Carcinoma 6 1% Rhabdomyosarcoma 2 Neuroblastoma 2 Fibrous histiocytoma 1 Acinic cell carcinoma 1 Histiocytosis X 1 Chloroma 1 Total 445

Torsiglieri et al., 19882


Inflammatory

lesions

27%

Malignant

neoplasms, 11%

Benign neoplasms

3%

Non-infammatory

benign lesions

5%

Congenital lesions

54%

N= 445

Initial Evaluation

H&P Age

Onset

Rapidity of growth

Fluctuation in size

Pain

Infection

Trauma

Travel

Exposure

PE Size

Multiplicity

Laterality

Consistency

Color

Mobility

Tenderness

Fluctuation

Congenital

Inflammatory

Benign

Malignant

Location, Location, Location!

Moir. 20048

Age of Distrubtion Range Average (years)

Brachial cleft cyst 6m – 16 y 3.6 y Thyroglossal duct cyst 9 m – 17 y 6.1 y Dermoid cyst 9 m – 15 y 3.7 y Lymphangioma 9m – 15 y 3.6 y Hemangioma 1 day – 15 y 5.6 y Reactive lymphadenopathy

3 m – 18 y 8.0 y

Graunlomatous disease

1 y – 14 y 6.0 y

Suppurative lymphadenitis

4 m – 15 y 7.3 y

Sialadenitis 11 y – 13 y 11.2 y Inclusion cyst 3 y – 12 y 4.4 y Fibriomatosis 1 m – 10 y 3.1 y Lymphoma 4 y – 21 y 11.7 y Thyroid Carcinoma 8 y – 17 y 12.3 y Others 2 weeks – 18 y 4.6 y


Likely Etiology Determines Direction of Testing

X-ray

U/S

CT

MRI

FNA

Surgical Biopsy

Tissue Culture

CXR

Labs

PPD

Gram stain

Culture


1. Congenital lesions

2. Inflammatory lesions

3. Non-inflammatory benign lesions

4. Benign neoplasms

5. Malignant neoplasms

1. Congenital Lesions

Branchial cleft cyst 78 18% Thyroglossal duct cyst 73 16% Dermoid cyst 43 10% Lymphangioma 34 8% Hemangioma 10 2% Teratoma 2 Bronchogenic cyst 2 Thymic cyst 1 Myelomeningocele 1

Embryology

Ectoderm, mesoderm, endoderm

Incomplete closure may result in branchial cleft anomalies

Moir. 20048

Development of First Four Arches

Nicollas. 20003

Each arch layer gives rise to:

•nerve (ectoderm)

•artery, muscle and cartilage (mesoderm)

•glands (endoderm).

Cyst Sinus Fistula

Schroeder. 20074

Branchial Cleft Anomalies

Distribution of neck malformations as cysts, fistulas, or sinuses

per Nicollas et. al. (n=191)

Nicollas. 20003

(Sinus)

Total 139 5 47 191

Moir. 20048

Imagining in Branchial Cleft Cysts

MRI

More reliably confirms cystic nature

More precisely defines lesion

Better to delineate glandular tissue

ie fat planes

CT

Adequate for most lesions

Cost, availability

U/S

cystic vs noncystic

does not evaluate extent

Both MRI and CT have difficulty distinguishing branchial cleft cyst from lymphangioma in children.

Branstetter, 20069

1st Branchial Cleft Cyst, Type II

Type I Ectodermal duplication of

EAC Near external auditory

canal Usually inferior and

posterior to tragus

Type II Associated with

submandibular gland

Branstetter, 20069

Type 1 First Branchial Cleft Cyst


Branstetter, 20069

Lymphangioma

mass

auricle

parotid

Branchial Cyst

Noncalcified mass

CT shows lesion under SCM

Malik et al, 20026

2nd Brachial Cleft Cyst T2 MRI

Posterior to R submandibular gland

Thickened walls suggest prior hemorrhage or infection

Gujar and Mukherji 20045

Anterior to carotid bifurcation

Schroeder et al, 20074

Under the anterior SCM


Exiting skin medial to lateral border of SCM


Left 2nd BA Fistula

Anterior to carotid bifurcation Under the anterior SCM Exiting skin medial to lateral border of SCM


Moir. 20048

2nd BA cyst with sinus tract extending into the pharynx above the carotid bifurcation


Watch the hypoglossal!

Preauricular Sinus

Not related to 1st branchial cleft anomalies

Active infection during excision increases chance of recurrance

Moir. 20048

Thyroglossal Duct Cyst

persistent tract from the descent of the thyroid from the foramen cecum

epithelial lining composed of either squamous or respiratory epithelium

confirm normal thyroid tissue

Learning Radiology.com 200711

Thyroglossal Duct Cyst

CT w/ Contrast

Embedded in the strap muscles

Extends deep to involve the pre-epiglottic space


Moir. 20048

Dermoid Cysts

Ectoderm and mesoderm

7% of dermoid cysts occur in head and neck

Thought to be of congenital inclusion type

mean diameter = 1.2 cm (0.6-3.3)

Treatment: complete excision

Pryor et al 200512

Dermoid Cysts – Cranial Theory

Grunwald in 1910

As neuroectodermal tract recedes, demal attachements follow its course and can form a sinus or cyst

Beware of possible intracranial involvement

Pryor et al 200512

•Orbit is the most common site for dermoids in the head and neck (61%)

•Direct excision is sufficient for neck dermoids, more extensive approaches (craniotomy, mastoidectomy) are needed for other sites

Diff dx: in midline of neck: thyroglossal duct cyst

in head & neck, n = 59

Pryor et al 200512

Dermoid Cysts

H & E

CT of dermoid cyst

Pryor et al 200512

Teratoma

H&N account for ~2% of teratomas

Newborn – 2.5 yr at presentation

All 3 germinal layers present

Mostly benign lesions amenable to curative excision

Wakhlu A et al 200013

Teratoma

• Prognosis good if no respiratory compromise

• Usually well differentiated and recurrence is uncommon

• Antenatal diagnosis is routine in developed world


Teratoma

• Proximity to vital structures makes surgery technically demanding.

• Evaluate post op thyroid and parathyroid function.


Teratoma – 3 germ layers

Arise from pluripotent cells and ectopic embryogenic non-germ cells


Teratoma – 3 germ layers


Hypopharyngeal Teratoma

calcified calcification and fat

Malik et al, 20026

Teratoma T1 MRI

Fatty

Calcified


Lymphangioma

Benign, multiloculated, soft

Posterior neck triangle predominance

Multi-septated, insinuating lesions

Infiltrate and cross tissue planes

Most occur by 2 yrs of age

Incidence: 1 in 6,000 to 16,000 births

Burezq 200614 Head and and Neck Surgery, 200615

Lymphatic Vascular malformation

T1 MRI

High signal represents proteinaceous fluid

Crosses tissue planes


Centrifugal vs Centripetal

Centrifugal theory

the lymphatic system develops as mesenchymal spaces that later coalesce into a system of vessels that eventually join the venous system.

Centripetal theory

jugular and posterior lymphatics form as outgrowths of endothelium from veins into the surrounding mesenchyme.

Burezq 200614

Classification

Size:

Microcystic: capillary lymphangiomas lesions are less than 1 cm in diameter

Macrocystic: cystic hygromas cysts are larger than 1 cm

Cystic hygromas #1 type of lymphangioma

Gross et al, 200616

Cystic Hygroma

Noncalcified

Septated on U/S

Malik et al, 20026

Cystic Composition

5-year-old boy with lymphangioma

L parotid & parapharyngeal space

mixed macro- andmicrocystic type

Treated by surgical resection

Gross et al, 200616

Type 1 First Branchial Cleft Cyst


Branstetter, 20069

Lymphangioma

mass

auricle

parotid

Burezq et al, 2006 (expert opinion)

1. Error in establishing a communication between the lymphatic and venous system

Cystic hygroma

2. Error in morphogenesis of lymphatic system: this includes other types of lymphatic malformations

microcystic, macrocystic and mixed lymphatic lesions

Burezq 200614

Management - Controversial

Spontaneous resolution? Formation of new lymphatic channels?

Serial aspiration?

Sclerosant Agents? OK-432 (lyophilizied mixture of low-virulence group A Sterp

pyogens

Surgical Excision? Is the surgical risk out weigh the benefit in a benign lesion

Burezq 200614

Success with Serial Aspirations

Burezq 200614

Success with OK-432

Supraclavicular macrocystic lymphangioma

Gross et al, 200616

Hemangioma

Less than 1/3 present at birth

Usually seen in 1st few months of life and enlarge progressively

90% cases involutes spontaneously

Sclerosing agents controversial

Glut-1

erythrocyte-type glucose transporter

found only in microvascular endothelia of blood–tissue barriers such as in the central nervous system, retina, placenta, ciliary muscle, and endoneurium of peripheral nerves

Hemangiomas stain consistently for Glut-1, in all stages of development and involution whereas vascular malformations did not

Mo et al, 200617

MacArther, 200618





4. Benign neoplasms


2. Inflammatory Lesions

Reactive lympadenopathy 71 16% Undetermined etiology 66 15% Sinus histiocytosis 5 1% Granulomatous disease 32 7% Atypical mycobacteria 20 4% Cat scratch disease 6 1% Toxoplasmosis 2 Sarcoid 2 Suppurative lympadenitis 10 2% Sialadenitis 5 1%

When does cervical lymphadenopathy require FNA?

Benign reactive lymph node may persist for weeks to months

Lymphoma can present the same way

Rapkiewicz et al 200721

To FNA or not to FNA?

Reactive lymphadenopathy the most likely etiology of pediatric neck masses

Diagnostic dilema: a mass that does not resolve after initial treatment


FNA ancillary studies

Gram stain, culture

Acid fast stain

Imunocytochemistry

Cytogenetics


Limitations to FNA

A lesion may not be homogenous

FNA samples only part of the mass

May miss the true lesion

Unable to appreciate histological architecture


Time to contemplate open biopsy

Enlarging mass

Poor response to medical treatment

Suspicious clinical course

Unusual image findings

Systemic symptoms


Case – F.R.

8 y/o female, hx + PPD several yrs prior

Presents with R cervical adenopathy

FNA suggests granuloma

Repeat FNA -> same result

AFB stain and cultures negative

Clarithromycin and ethambutol started


Case – F.R.

Adenopathy and pain increased

Third FNA non-diagnostic

CT shows bulky homogenous lymphadenopathy of R upper spinal accessory and upper jugular chains.

Open biopsy displayed Hodgkin's lymphoma.


Reactive Lymphadenopathy

3-year-old child

Multiple hypoechoic lesions

variable shape and sizes

consistent with reactive lymph nodes

Malik et al, 20026

Enlarged Lymph Node

Nonspecific

Cause:

cryptococcal adenitis

Yeastlike fungus


Atypical mycobacteria: ex: cryptococcus

Saprobe in nature

worldwide distribution

Found in soil

Portal of entry is lung

Atypical mycobacteria: ex: cryptococcus

Associated w/ AIDS

organ transplantation

Lymphoreticular diseases

½ pts lack apprarent predisposing factors

Bartonella henselae

Hypoechoic masses with irregular rim of isoechoic tissue

Biopsy: Cat Scratch Disease

Malik et al, 20026

Bartonella henselae

Gram – coccobacillus

2- 14 day incubation

Dx: requires prolonged incubation (2 + weeks)

Rx: erythromycin 1-4 m (unclear efficacy)

Normally benign course

Malik et al, 20026

In heart valve

Peritonsillar Abcess

Soft tissue density in submental space

Malik et al, 20026

Retropharyngeal Abscess

Widening of prevertebral space

Malik et al, 20026

Retropharyngeal Space Abscess


Retropharyngeal Peritonsillar

Malik et al, 20026

Sppurative Cervical Adenitis

hypoechoic

Malik et al, 20026

Thyroid Abscess

Malik et al, 20026





4. Benign neoplasms


3. Non-inflammatory Benign Lesions

Inclusion cyst 13 3% Fibromatosis 9 2% Keloid 1

Inclusion Cyst

Acquired dermoid cysts result from a part of the skin being traumatically

implanted in the deeper layers after ectopic formation of a dermal cyst lined with squamous epithelium.

Congenital inclusion dermoid cysts form along the lines of embryologic fusion and contain both dermal and epidermal derivatives. Dermoid cysts of the head and neck are thought to be

the congenital inclusion type.

Pryor et al 200512

Inclusion Cyst

many cysts originate from the infundibular portion of the hair follicle, and the more general term, epidermoid cyst, is favored

Becker et a, 200519

Epidermal Inclusion Cyst

Cyst containing keratinous material true epidermis with a granular layer and adjacent laminated keratinous material

Becker et al, 200519

Torticollis

Fibromatosis Colli

SCM

Isoechoic mass

CT shows isodense mass R side

Note normal SCM on L side

Malik et al, 20026

Fibromatosis Colli - FNA

Paucicellular specimen

Bland spindle cell cytology

r/o nodular fascitis and fibrosarcoma


Fibromatosis Colli

r/o nodular fascitis and fibrosarcoma

Roy, 200720

Moir. 20042





4. Benign neoplasms


4. Benign Neoplasms

Neurofibroma 3 1% Lipoma 3 1% Lipoblastoma 2 Paraganglioma 1 Goiter 1 Benign mixed tumor 1 Osteoblastoma 1

Neurofibroma

solitary lesion

vs

part of the generalized syndrome of neurofibromatosis NF-1, aka von Recklinghausen disease

NF-2

Believed to arise from Schwann cell but origin uncertain

Neurofibroma

T2 MRI

Central low T2 signal is characteristic of neurofibromas


Lipoma

http://www.microscopyu.com/galleries/pathology/lipomalarge.html

Lipoblastoma

Rare benign mesynchymal tumor of embryonal fat

May clinically and radiologically mimic a hemangioma

Collections of lipoblasts – multivuolated w/ round nuclei

FNA

Lipoblastoma

Resembles embryological adipose tissue

Surgical specimen

Lipoma Lipoblastoma

http://www.microscopyu.com/galleries/pathology/lipomalarge.html

Neonatal Goiter

CT shows large peripheral rim enhancing, low attenuation mass

1: 4000 live births

Female 2x = Male predominance

Delayed ossification at bone ends

Malik et al, 20026

Rovet et al, 200310





4. Benign neoplasms


5. Malignant Neoplasms

Lymphoma 34 8% Hodgkin's 23 5% Non-Hodkin's 11 2% Thyroid Carcinoma 6 1% Rhabdomyosarcoma 2 Neuroblastoma 2 Fibrous histiocytoma 1 Acinic cell carcinoma 1 Histiocytosis X 1 Chloroma 1

Lymphoma

Third most common pediatric cancer

Incidence: 11-20 per million children

Geographical variance – 50 % of childhood cancers in equatorial Africa

Due to high incidence of Burkitt’s lymphoma

Male predominance 2.5:1

Beware the supraclavicular mass!

35% of patients with H&N lymphoma present with a supraclavicular mass

35% of pts with suprclavicular masses had lymphoma

Turkington et al 200522 Torsiglieri et al., 19882

Neuroblastoma

Noncontrast T1 MRI

Mass (arrow) lateral to carotid artery (arrowhead).


Rhabdomyosarcoma - CT

Ill defined

enhancing soft tissue density

areas of necrosis

Malik et al, 20026

Rhabdomyosarcoma of the Masticator Space

Non-contrast T1 – intermediate signal

T2 – increased signal



Inflammatory

lesions

27%

Malignant

neoplasms, 11%

Benign neoplasms

3%

Non-infammatory

benign lesions

5%

Congenital lesions

54%

N= 445

Total % of total

Congeital lesions 244 55% Branchial cleft cyst 78 18% Thyroglossal duct cyst 73 16% Dermoid cyst 43 10% Lymphangioma 34 8% Hemangioma 10 2% Teratoma 2 Bronchogenic cyst 2 Thymic cyst 1 Myelomeningocele 1 Inflammatory lesions 118 27% Reactive lympadenopathy 71 16% Undetermined etiology 66 15% Sinus histiocytosis 5 1% Granulomatous disease 32 7% Atypical mycobacteria 20 4% Cat scratch disease 6 1% Toxoplasmosis 2 Sarcoid 2 Suppurative lympadenitis 10 2% Sialadenitis 5 1%

Non-infammatory benign lesions

23 5%

Inclusion cyst 13 3% Fibromatosis 9 2% Keliod 1 Benign neoplasms 12 3% Neurofibroma 3 1% Lipoma 3 1% Lipoblastoma 2 Paraganglioma 1 Goiter 1 Benign mixed tumor 1 Osteoblastoma 1 Malignant neoplasms 48 11% Lymphoma 34 8% Hodgkin's 23 5% Non-Hodkin's 11 2% Thyroid Carcinoma 6 1% Rhabdomyosarcoma 2 Neuroblastoma 2 Fibrous histiocytoma 1 Acinic cell carcinoma 1 Histiocytosis X 1 Chloroma 1 Total 445


Conclusions

Initial evaluation (H&P) Congenital, infectious, benign, malignant

Beware of tuberculosis, cat scratch disease, atypical infections

Beware of systemic symptoms

Beware the supraclavicular mass

Consider FNA or biopsy in the mass that does not resolve with treatment.

Bibliography

1. NeoReviews.org, http://neoreviews.aappublications.org/case27/case.shtml, 10/18/07.

2. Torsiglieri AJ Jr, Tom LW, Ross AJ 3rd, Wetmore RF, Handler SD, Potsic WP. Pediatric neck masses: guidelines for evaluation. Int J Pediatr Otorhinolaryngol. 1988 Dec;16(3):199-210.

3. Nicollas R, Guelfucci B, Roman S, Triglia JM. Congenital cysts and fistulas of the neck. Int J Pediatr Otorhinolaryngol. 2000 Sep 29;55(2):117-24.

4. Schroeder JW Jr, Mohyuddin N, Maddalozzo J. Branchial anomalies in the pediatric population. Otolaryngol Head Neck Surg. 2007 Aug;137(2):289-95.

5. Gujar S, Gandhi D, Mukherji SK. Pediatric head and neck masses. Top Magn Reson Imaging. 2004 Apr;15(2):95-101.

6. Malik A, Odita J, Rodriguez J, Hardjasudarma M. Pediatric neck masses: a pictorial review for practicing radiologists. Curr Probl Diagn Radiol. 2002 Jul-Aug;31(4):146-57.

http://neoreviews.aappublications.org/case27/case.shtml

Bibliography (cont)

7. ROH, JL.Lymphomas of the head and neck in the pediatric population, International journal of pediatric otorhinolaryngology, Volume 71, Issue 9, September 2007, Pages 1471-1477.

8. Moir CR. Neck Cysts, Sinuses, Thyroglossal Duct Cyts, and Branchial Cleft Anomalies, Operative Tech in Gen Surg, v 6, n 4 (Dec), 2004: 281-295.

9. Branstetter BF, Branchial Cleft Cysts, Emedicine, http://www.emedicine.com/radio/topic107.htm Oct 24, 2006.

10. Rovet JF. Congenital hypothyroidism: an analysis of persisting deficits and associated factors. Child Neuropsychol. 2002 Sep;8(3):150-62.

11. Thyroglossal Duct Cyst, Learning Radiology.com, http://www.learningradiology.com/archives06/COW%20231-Thyroglossal%20Duct%20Cyst/tgdccorrect.html, accessed 10/30/2007.

http://www.emedicine.com/radio/topic107.htm

http://www.learningradiology.com/archives06/COW 231-Thyroglossal Duct Cyst/tgdccorrect.html



Bibliography (cont)

12. Pryor SG, Lewis JE, Weaver AL, Orvidas LJ. Pediatric dermoid cysts of the head and neck. Otolaryngol Head Neck Surg. 2005 Jun;132(6):938-42.

13. Wakhlu A, Wakhlu AK. Head and neck teratomas in children. Pediatr Surg Int. 2000;16(5-6):333-7. 14. Burezq: J Craniofac Surg, Management of Cystic Hygromas: 30 Year Experience Volume 17(4).July 2006.815-

818. 15. Head and Neck Surgery—Otolaryngology, Bailey,Calhoun, 2006, p.1213-1215 16. Gross E, Sichel JY. Congenital neck lesions. Surg Clin North Am. 2006 Apr;86(2):383-92, ix. 17. Mo JQ, Dimashkieh HH, Bove KE, GLUT1 endothelial reactivity distinguishes hepatic infantile hemangioma

from congenital hepatic vascular malformation with associated capillary proliferation. Hum Pathol. 2004 Feb;35(2):200-9.

18. MacArthur CJ , Head and neck hemangiomas of infancy. Current opinion in otolaryngology & head and neck surgery, 12/2006, Vol: 14, Issue: 6 Page: 397.

19. Becker KA, Thomas I. Epidermal Inclusion Cyst. Emedicine.com 5/10/2006. www.emedicine.com/derm/topic860.htm

20. Roy S, Fibromatosis Colli, Histopathology India.net www.histopathology-india.net/FC.htm 21. Rapkiewicz A, Le BT, Simsir A, Cangiarella J, Levine P. Spectrum of head and neck lesions diagnosed by fine-

needle aspiration cytology in the pediatric population. Cancer Cytopathology. Vol 111, Issue 4, Pages 242-251, 6 Jun 2007.

22. J R A Turkington, A Paterson, L E Sweeney, G D Thornbury. Neck Masses in Childres. BR J of Radiology, 78 (2005), 75-85.

http://www.emedicine.com/derm/topic860.htm

http://www.histopathology-india.net/FC.htm



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