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 Congeital lesions Branchial cleft cyst Thyroglossal duct cyst Dermoid cyst Lymphangioma Hemangioma Teratoma Bronchogenic cyst Thymic cyst Myelomeningocele Inflammatory lesions Reactive lympadenopathy Undetermined etiology Sinus histiocytosis Granulomatous disease Atypical mycobacteria Cat scratch disease Toxoplasmosis Sarcoid Suppurative lympadenitis Sialadenitis 244 78 73 43 34 10 2 2 1 1

% of total 55% 18% 16% 10% 8% 2%

Non-infammatory benign lesions Inclusion cyst Fibromatosis Keliod Benign neoplasms Neurofibroma Lipoma Lipoblastoma Paraganglioma Goiter Benign mixed tumor Osteoblastoma Malignant neoplasms Lymphoma Hodgkin's Non-Hodkin's Thyroid Carcinoma Rhabdomyosarcoma Neuroblastoma Fibrous histiocytoma Acinic cell carcinoma Histiocytosis X Chloroma Total

23 13 9 1 12 3 3 2 1 1 1 1

5% 3% 2%

3% 1% 1%

118 27% 71 16% 66 15% 5 1% 32 7% 20 4% 6 1% 2 2 10 2% 5 1%

Torsiglieri et al., 19882

48 11% 34 8% 23 5% 11 2% 6 1% 2 2 1 1 1 1 445

Torsiglieri et al.,
Malignant neoplasms, 11% Benign neoplasms 3% Non-infammatory benign lesions 5%

2 1988

Congenital lesions 54% Inflammatory lesions 27%

N= 445

Congenital

Benign

Inflammatory

Initial Evaluation
Malignant

H&P
Age Onset Rapidity of growth Fluctuation in size Pain Infection Trauma Travel Exposure

PE
Size Multiplicity Laterality Consistency Color Mobility Tenderness Fluctuation

Location, Location, Location!

Moir. 20048

Age of Distrubtion
Range Brachial cleft cyst Thyroglossal duct cyst Dermoid cyst Lymphangioma Hemangioma Reactive lymphadenopathy Graunlomatous disease Suppurative lymphadenitis Sialadenitis Inclusion cyst Fibriomatosis Lymphoma Thyroid Carcinoma Others 6m 16 y 9 m 17 y 9 m 15 y 9m 15 y 1 day 15 y 3 m 18 y 1 y 14 y 4 m 15 y 11 y 13 y 3 y 12 y 1 m 10 y 4 y 21 y 8 y 17 y 2 weeks 18 y Average (years) 3.6 y 6.1 y 3.7 y 3.6 y 5.6 y 8.0 y 6.0 y 7.3 y 11.2 y 4.4 y 3.1 y 11.7 y 12.3 y 4.6 y

Torsiglieri et al., 19882

Likely Etiology Determines Direction of Testing

X-ray U/S CT MRI FNA Surgical Biopsy Tissue Culture CXR Labs PPD Gram stain Culture

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

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

Embryology
Ectoderm, mesoderm, endoderm Incomplete closure may result in branchial cleft

anomalies

Moir. 20048

Development of First Four Arches

Each arch layer gives rise to: nerve (ectoderm) artery, muscle and cartilage (mesoderm) glands (endoderm).

Nicollas. 20003

Branchial Cleft Anomalies

Cyst Sinus Fistula

Schroeder. 20074

Distribution of neck malformations as cysts, fistulas, or sinuses

per Nicollas et. al. (n=191)
(Sinus)

Total

139

47

191

Nicollas. 20003

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 Branstetter

st 1

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 Branstetter

Lymphangioma Type 1 First Branchial Cleft Cyst

mass

Both MRI and CT have difficulty distinguishing branchial cleft cyst from lymphangioma in children.
auricle parotid Branstetter, 20069 Branstetter

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 Schroeder et al, 20074

Exiting skin medial to lateral border of SCM Schroeder et al, 20074

Left

nd 2

BA Fistula

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

Schroeder et al, 20074

Moir. 20048

Moir. 20048

Moir. 20048

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

Schroeder et al, 20074

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 preepiglottic space

Gujar and Mukherji 20045

Moir. 20048

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

in head & neck, n = 59

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 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
Wakhlu A et al 200013

Teratoma
Proximity to vital structures makes surgery technically demanding. Evaluate post op thyroid and parathyroid function.

Wakhlu A et al 200013

Teratoma 3 germ layers

Arise from pluripotent cells and ectopic embryogenic non-germ cells

Wakhlu A et al 200013

Teratoma 3 germ layers

Wakhlu A et al 200013

Teratoma 3 germ layers

Wakhlu A et al 200013

Hypopharyngeal Teratoma

calcified

calcification and fat

Malik et al, 20026

Teratoma
T1 MRI

Calcified

Fatty

Gujar and Mukherji 20045

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

Head and and Neck Surgery, 200615

Burezq 200614

Lymphatic Vascular malformation

T1 MRI High signal represents proteinaceous fluid Crosses tissue planes
Gujar and Mukherji 20045

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 macroandmicrocystic type Treated by surgical resection
Gross et al, 200616

Lymphangioma Type 1 First Branchial Cleft Cyst

mass

Both MRI and CT have difficulty distinguishing branchial cleft cyst from lymphangioma in children.
auricle parotid Branstetter, 20069 Branstetter

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 bloodtissue 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
MacArther, 200618 Mo et al, 200617

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

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

Rapkiewicz et al 200721

FNA ancillary studies

Gram stain, culture Acid fast stain Imunocytochemistry Cytogenetics

Rapkiewicz et al 200721

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

Rapkiewicz et al 200721

Time to contemplate open biopsy

Enlarging mass Poor response to medical treatment Suspicious clinical course Unusual image findings Systemic symptoms

Rapkiewicz et al 200721

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

Rapkiewicz et al 200721

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.
Rapkiewicz et al 200721

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

Gujar and Mukherji 20045

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

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

In heart valve

Malik et al, 20026

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

Gujar and Mukherji 20045

Peritonsillar

Retropharyngeal

Malik et al, 20026

Sppurative Cervical Adenitis

hypoechoic Malik et al, 20026

Thyroid Abscess

Malik et al, 20026

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

3. Non-inflammatory Benign Lesions

Inclusion cyst Fibromatosis Keloid 13 9 1 3% 2%

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
Rapkiewicz et al 200721

Fibromatosis Colli

r/o nodular fascitis and fibrosarcoma

Roy, 200720

Moir. 20042

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

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

Gujar and Mukherji 20045

Lipoma

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

Neonatal Goiter
CT shows large peripheral rim enhancing, low attenuation mass 1: 4000 live births Female 2x = Male predominance Delayed ossification at bone ends
Rovet et al, 200310 Malik et al, 20026

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

Pediatric Neck Masses

1. 2. 3. 4. 5. Congenital lesions Inflammatory lesions Non-inflammatory benign lesions Benign neoplasms Malignant neoplasms

5. Malignant Neoplasms
Lymphoma Hodgkin's Non-Hodkin's Thyroid Carcinoma Rhabdomyosarcoma Neuroblastoma Fibrous histiocytoma Acinic cell carcinoma Histiocytosis X Chloroma 34 23 11 6 2 2 1 1 1 1 8% 5% 2% 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 Burkitts 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).
Gujar and Mukherji 20045

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
Gujar and Mukherji 20045

Torsiglieri et al.,
Malignant neoplasms, 11% Benign neoplasms 3% Non-infammatory benign lesions 5%

2 1988

Congenital lesions 54% Inflammatory lesions 27%

N= 445

Total Congeital lesions Branchial cleft cyst Thyroglossal duct cyst Dermoid cyst Lymphangioma Hemangioma Teratoma Bronchogenic cyst Thymic cyst Myelomeningocele Inflammatory lesions Reactive lympadenopathy Undetermined etiology Sinus histiocytosis Granulomatous disease Atypical mycobacteria Cat scratch disease Toxoplasmosis Sarcoid Suppurative lympadenitis Sialadenitis 244 78 73 43 34 10 2 2 1 1

% of total 55% 18% 16% 10% 8% 2%

Non-infammatory benign lesions Inclusion cyst Fibromatosis Keliod Benign neoplasms Neurofibroma Lipoma Lipoblastoma Paraganglioma Goiter Benign mixed tumor Osteoblastoma Malignant neoplasms Lymphoma Hodgkin's Non-Hodkin's Thyroid Carcinoma Rhabdomyosarcoma Neuroblastoma Fibrous histiocytoma Acinic cell carcinoma Histiocytosis X Chloroma Total

23 13 9 1 12 3 3 2 1 1 1 1

5% 3% 2%

3% 1% 1%

118 27% 71 16% 66 15% 5 1% 32 7% 20 4% 6 1% 2 2 10 2% 5 1%

Torsiglieri et al., 19882

48 11% 34 8% 23 5% 11 2% 6 1% 2 2 1 1 1 1 445

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.

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