Pediatric Neck Masses

1
Pediatric Neck Masses

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

1
2
Torsiglieri et al., 19882
3
Torsiglieri et al., 19882
N 445
4
Initial Evaluation
Congenital
Benign
Inflammatory
Malignant

• HP
• Age
• Onset
• Rapidity of growth
• Fluctuation in size
• Pain
• Infection
• Trauma
• Travel
• Exposure

• PE
• Size
• Multiplicity
• Laterality
• Consistency
• Color
• Mobility
• Tenderness
• Fluctuation

5
Location, Location, Location!
Moir. 20048
6
Age of Distrubtion
Torsiglieri et al., 19882
7
Likely Etiology Determines Direction of Testing

• X-ray
• U/S
• CT
• MRI

• FNA
• Surgical Biopsy
• Tissue Culture
• CXR
• Labs
• PPD
• Gram stain
• Culture

8
Pediatric Neck Masses

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

9
Pediatric Neck Masses

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

10
1. Congenital Lesions
11
Embryology

• Ectoderm, mesoderm, endoderm
• Incomplete closure may result in branchial cleft
  anomalies

Moir. 20048
12
Development of First Four Arches

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

Nicollas. 20003
13
Branchial Cleft Anomalies
Cyst
Sinus
Fistula
Schroeder. 20074
14
Distribution of neck malformationsas cysts,
fistulas, or sinusesper Nicollas et. al. (n191)
(Sinus)
Total 139 5 47 191
Nicollas. 20003
15
Moir. 20048
16
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
17
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
18
Type 1 First Branchial Cleft Cyst
Lymphangioma
mass

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

auricle
parotid
Branstetter, 20069
19
Branchial Cyst

• Noncalcified mass
• CT shows lesion under SCM

Malik et al, 20026
20
2nd Brachial Cleft CystT2 MRI

• Posterior to R submandibular gland
• Thickened walls suggest prior hemorrhage or
  infection

Gujar and Mukherji 20045
21

• Anterior to carotid bifurcation

Schroeder et al, 20074
22

• Under the anterior SCM

Schroeder et al, 20074
23

• Exiting skin medial to lateral border of SCM

Schroeder et al, 20074
24
Left 2nd BA Fistula

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

Schroeder et al, 20074
25
Moir. 20048
26
Moir. 20048
27
Moir. 20048
28

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

Watch the hypoglossal!
Schroeder et al, 20074
29
Preauricular Sinus

• Not related to 1st branchial cleft anomalies
• Active infection during excision increases chance
  of recurrance

Moir. 20048
30
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
31
Thyroglossal Duct Cyst

• CT w/ Contrast
• Embedded in the strap muscles
• Extends deep to involve the pre-epiglottic space

Gujar and Mukherji 20045
32
Moir. 20048
33
Moir. 20048
34
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
35
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
36
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
37
Dermoid Cysts
H E
CT of dermoid cyst
Pryor et al 200512
38
Teratoma

• HN 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
39
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
40
Teratoma

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

Wakhlu A et al 200013
41
Teratoma 3 germ layers

• Arise from pluripotent cells and ectopic
  embryogenic non-germ cells

Wakhlu A et al 200013
42
Teratoma 3 germ layers
Wakhlu A et al 200013
43
Teratoma 3 germ layers
Wakhlu A et al 200013
44
Hypopharyngeal Teratoma
calcified
calcification and fat
Malik et al, 20026
45
TeratomaT1 MRI
Calcified
Fatty
Gujar and Mukherji 20045
46
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
47
Lymphatic Vascular malformation

• T1 MRI
• High signal represents proteinaceous fluid
• Crosses tissue planes

Gujar and Mukherji 20045
48
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
49
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
50
Cystic Hygroma

• Noncalcified
• Septated on U/S

Malik et al, 20026
51
Cystic Composition

• 5-year-old boy with lymphangioma
• L parotid parapharyngeal space
• mixed macro- andmicrocystic type
• Treated by surgical resection

Gross et al, 200616
52
Type 1 First Branchial Cleft Cyst
Lymphangioma
mass

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

auricle
parotid
Branstetter, 20069
53
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
54
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
55
Success with Serial Aspirations
Burezq 200614
56
Success with OK-432
Supraclavicular macrocystic lymphangioma
Gross et al, 200616
57
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

58
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
59
Pediatric Neck Masses

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

60
Pediatric Neck Masses

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

61
2. Inflammatory Lesions
62
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
63
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
64
FNA ancillary studies

• Gram stain, culture
• Acid fast stain
• Imunocytochemistry
• Cytogenetics

Rapkiewicz et al 200721
65
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
66
Time to contemplate open biopsy

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

Rapkiewicz et al 200721
67
Case F.R.

• 8 y/o female, hx PPD several yrs prior
• Presents with R cervical adenopathy
• FNA suggests granuloma
• Repeat FNA -gt same result
• AFB stain and cultures negative
• Clarithromycin and ethambutol started

Rapkiewicz et al 200721
68
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
69
Reactive Lymphadenopathy

• 3-year-old child
• Multiple hypoechoic lesions
• variable shape and sizes
• consistent with reactive lymph nodes

Malik et al, 20026
70
Enlarged Lymph Node

• Nonspecific
• Cause
• cryptococcal adenitis
• Yeastlike fungus

Gujar and Mukherji 20045
71
Atypical mycobacteria ex cryptococcus

• Saprobe in nature
• worldwide distribution
• Found in soil
• Portal of entry is lung

72
Atypical mycobacteria ex cryptococcus

• Associated w/
• AIDS
• organ transplantation
• Lymphoreticular diseases
• ½ pts lack apprarent predisposing factors

73
Bartonella henselae

• Hypoechoic masses with irregular rim of isoechoic
  tissue
• Biopsy Cat Scratch Disease

Malik et al, 20026
74
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
75
Peritonsillar Abcess

• Soft tissue density in submental space

Malik et al, 20026
76
Retropharyngeal Abscess

• Widening of prevertebral space

Malik et al, 20026
77
Retropharyngeal Space Abscess
Gujar and Mukherji 20045
78
Retropharyngeal
Peritonsillar
Malik et al, 20026
79
Sppurative Cervical Adenitis
hypoechoic
Malik et al, 20026
80
Thyroid Abscess
Malik et al, 20026
81
Pediatric Neck Masses

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

82
Pediatric Neck Masses

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

83
3. Non-inflammatory Benign Lesions
84
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
85
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
86
Inclusion Cyst

• Discharge of a foul-smelling cheeselike material
  is a common complaint.
• Less frequently, the cysts can become inflamed or
  infected, resulting in pain and tenderness.
• In the uncommon event of malignancy, rapid
  growth, friability, and bleeding have been
  reported
• Injuries, especially of the crushing type, such
  as the slamming of a car door on a finger, are
  frequently reported in association with subungual
  or terminal phalanx epidermoid cysts.
• Theoretically, any surgical procedure may result
  in epidermoid cysts, and it is surprising that
  they are not a more common occurrence

Becker et al, 200519
87
Epidermal Inclusion Cyst
Cyst containing keratinous material
true epidermis with a granular layer and adjacent
laminated keratinous material
Becker et al, 200519
88
Torticollis
89
Fibromatosis Colli

• SCM
• Isoechoic mass
• CT shows isodense mass R side
• Note normal SCM on L side

Malik et al, 20026
90
Fibromatosis Colli - FNA

• Paucicellular specimen
• Bland spindle cell cytology

• r/o nodular fascitis and fibrosarcoma

Rapkiewicz et al 200721
91
Fibromatosis Colli

• r/o nodular fascitis and fibrosarcoma

Roy, 200720
92
Moir. 20042
93
Pediatric Neck Masses

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

94
Pediatric Neck Masses

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

95
4. Benign Neoplasms
96
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

97
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

98
Neurofibroma

• T2 MRI
• Central low T2 signal is characteristic of
  neurofibromas

Gujar and Mukherji 20045
99
Lipoma
100
Lipoblastoma

• Rare benign mesynchymal tumor of embryonal fat
• May clinically and radiologically mimic a
  hemangioma
• Collections of lipoblasts multivuolated w/
  round nuclei

FNA
101
Lipoblastoma

• Resembles embryological adipose tissue

Surgical specimen
102
Lipoma Lipoblastoma
103
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
104
Pediatric Neck Masses

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

105
Pediatric Neck Masses

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

106
5. Malignant Neoplasms
107
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 2.51

108
Beware the supraclavicular mass!

• 35 of patients with HN lymphoma present with a
  supraclavicular mass
• 35 of pts with suprclavicular masses had lymphoma

Torsiglieri et al., 19882
Turkington et al 200522
109
Roh et al 20077
110
Neuroblastoma

• Noncontrast T1 MRI
• Mass (arrow) lateral to carotid artery
  (arrowhead).

Gujar and Mukherji 20045
111
Rhabdomyosarcoma - CT

• Ill defined
• enhancing soft tissue density
• areas of necrosis

Malik et al, 20026
112
Rhabdomyosarcoma of the Masticator Space

• Non-contrast T1 intermediate signal
• T2 increased signal

Gujar and Mukherji 20045
113
Torsiglieri et al., 19882
N 445
114
Torsiglieri et al., 19882
115
Conclusions

• Initial evaluation (HP)
• 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.

116
Bibliography

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117
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