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% Non-infammatory benign 23 5%
Branchial cleft cyst 78 18% lesions
Thyroglossal duct cyst 73 16% Inclusion cyst 13 3%
Dermoid cyst 43 10% Fibromatosis 9 2%
Lymphangioma 34 8% Keliod 1
Hemangioma 10 2%
Teratoma 2 Benign neoplasms 12 3%
Bronchogenic cyst 2 Neurofibroma 3 1%
Thymic cyst 1 Lipoma 3 1%
Myelomeningocele 1 Lipoblastoma 2
Paraganglioma 1
Inflammatory lesions 118 27% Goiter 1
Reactive lympadenopathy 71 16% Benign mixed tumor 1
Undetermined etiology 66 15% Osteoblastoma 1
Sinus histiocytosis 5 1%
Granulomatous disease 32 7% Malignant neoplasms 48 11%
Atypical mycobacteria 20 4% Lymphoma 34 8%
Cat scratch disease 6 1% Hodgkin's 23 5%
Toxoplasmosis 2 Non-Hodkin's 11 2%
Sarcoid 2 Thyroid Carcinoma 6 1%
Suppurative lympadenitis 10 2% Rhabdomyosarcoma 2
Sialadenitis 5 1% Neuroblastoma 2
Fibrous histiocytoma 1
Acinic cell carcinoma 1
Histiocytosis X 1
Chloroma 1
Torsiglieri et al., 19882 Total 445
 Torsiglieri et al., 19882
Malignant
neoplasms, 11%
Benign neoplasms
3%
Non-infammatory
benign lesions
5%

Congenital lesions
54%
Inflammatory
lesions
27%

N= 445
 Congenital Benign

Initial Evaluation
Inflammatory
Malignant

„ H&P „ PE
„ Age „ Size
„ Onset „ Multiplicity
„ Rapidity of growth „ Laterality
„ Fluctuation in size „ Consistency
„ Pain „ Color
„ Infection „ Mobility
„ Trauma „ Tenderness
„ Travel „ Fluctuation
„ Exposure
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 3 m – 18 y 8.0 y
lymphadenopathy
Graunlomatous 1 y – 14 y 6.0 y
disease
Suppurative 4 m – 15 y 7.3 y
lymphadenitis
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

Torsiglieri et al., 19882
Likely Etiology Determines Direction
of Testing
„ X-ray „ FNA
„ U/S „ Surgical Biopsy
„ CT „ Tissue Culture
„ MRI
„ CXR
„ Labs
„ PPD
„ Gram stain
„ Culture
 Pediatric Neck Masses

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

„ 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

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 5 47 191

Nicollas. 20003
Moir. 20048
 Imagining in Branchial Cleft Cysts

MRI CT
„ More reliably confirms „ Adequate for most
cystic nature lesions
„ More precisely defines „ Cost, availability
lesion
„ Better to delineate U/S
glandular tissue „ cystic vs noncystic
„ ie fat planes
„ does not evaluate
extent
Both MRI and CT have difficulty distinguishing branchial
cleft cyst from lymphangioma in children.

Branstetter,
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,
Branstetter 20069
 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,
Branstetter 20069
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 2nd 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 pre-
epiglottic 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 macro-
andmicrocystic 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,
Branstetter 20069
 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
MacArther, 200618
Mo et al, 200617
 Pediatric Neck Masses

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

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

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
„ 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)
In heart valve
„ Normally benign course

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. Congenital lesions
„ 2. Inflammatory lesions
„ 3. Non-inflammatory benign lesions
„ 4. Benign neoplasms
„ 5. Malignant neoplasms
 Pediatric Neck Masses

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

Rapkiewicz et al 200721
 Fibromatosis Colli

„ r/o nodular fascitis and fibrosarcoma

Roy, 200720
Moir. 20042
 Pediatric Neck Masses

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

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

„ 1. Congenital lesions
„ 2. Inflammatory lesions
„ 3. Non-inflammatory benign lesions
„ 4. Benign neoplasms
„ 5. Malignant 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
Torsiglieri et al., 19882
Turkington et al 200522
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., 19882
Malignant
neoplasms, 11%
Benign neoplasms
3%
Non-infammatory
benign lesions
5%

Congenital lesions
54%
Inflammatory
lesions
27%

N= 445
 Total % of
total
Congeital lesions 244 55% Non-infammatory benign 23 5%
Branchial cleft cyst 78 18% lesions
Thyroglossal duct cyst 73 16% Inclusion cyst 13 3%
Dermoid cyst 43 10% Fibromatosis 9 2%
Lymphangioma 34 8% Keliod 1
Hemangioma 10 2%
Teratoma 2 Benign neoplasms 12 3%
Bronchogenic cyst 2 Neurofibroma 3 1%
Thymic cyst 1 Lipoma 3 1%
Myelomeningocele 1 Lipoblastoma 2
Paraganglioma 1
Inflammatory lesions 118 27% Goiter 1
Reactive lympadenopathy 71 16% Benign mixed tumor 1
Undetermined etiology 66 15% Osteoblastoma 1
Sinus histiocytosis 5 1%
Granulomatous disease 32 7% Malignant neoplasms 48 11%
Atypical mycobacteria 20 4% Lymphoma 34 8%
Cat scratch disease 6 1% Hodgkin's 23 5%
Toxoplasmosis 2 Non-Hodkin's 11 2%
Sarcoid 2 Thyroid Carcinoma 6 1%
Suppurative lympadenitis 10 2% Rhabdomyosarcoma 2
Sialadenitis 5 1% Neuroblastoma 2
Fibrous histiocytoma 1
Acinic cell carcinoma 1
Histiocytosis X 1
Chloroma 1
Torsiglieri et al., 19882 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.
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