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Pediatric Neck MassesPediatric Neck Masses

Mark Mark DomanskiDomanski, M.D., M.D.Michael Michael UnderbrinkUnderbrink, M.D., M.D.Dept. of Otolaryngology Dept. of Otolaryngology University of Texas Medical University of Texas Medical Branch, GalvestonBranch, GalvestonOctober 31October 31stst, 2007 , 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 1Total 445TorsiglieriTorsiglieri et al., 1988et al., 198822

TorsiglieriTorsiglieri et al., 1988et al., 198822

Inflammatory lesions

27%

Malignant neoplasms, 11%

Benign neoplasms3%

Non-infammatory benign lesions

5%

Congenital lesions54%

N= 445

Initial EvaluationInitial Evaluation

H&PH&PAgeAgeOnsetOnsetRapidity of growthRapidity of growthFluctuation in sizeFluctuation in sizePainPainInfectionInfectionTraumaTraumaTravelTravelExposureExposure

PEPESizeSizeMultiplicityMultiplicityLateralityLateralityConsistencyConsistencyColorColorMobilityMobilityTendernessTendernessFluctuationFluctuation

Congenital

Inflammatory

Benign

Malignant

Location, Location, Location!Location, Location, Location!

Moir. 20048

Age of Age of DistrubtionDistrubtion 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

TorsiglieriTorsiglieri et al., 1988et al., 198822

Likely Etiology Determines Direction Likely Etiology Determines Direction of Testingof Testing

XX--rayrayU/SU/SCTCTMRIMRI

FNAFNASurgical BiopsySurgical BiopsyTissue CultureTissue Culture

CXRCXRLabsLabsPPDPPDGram stainGram stainCultureCulture

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

1. Congenital Lesions1. 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

EmbryologyEmbryology

Ectoderm, Ectoderm, mesoderm, mesoderm, endodermendoderm

Incomplete closure Incomplete closure may result in may result in branchial cleft branchial cleft anomaliesanomalies

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 Branchial Cleft AnomaliesAnomalies

Distribution of neck malformationsas cysts, fistulas, or sinuses

per Nicollas et. al. (n=191)

Nicollas. 20003

(Sinus)

Total 139 5 47 191

Moir. 20048

Imagining in Branchial Cleft CystsImagining in Branchial Cleft Cysts

MRIMRIMore reliably confirms More reliably confirms cystic naturecystic natureMore precisely defines More precisely defines lesionlesionBetter to delineate Better to delineate glandular tissueglandular tissue

ieie fat planesfat planes

CTCTAdequate for most Adequate for most lesionslesionsCost, availabilityCost, availability

U/SU/Scystic cystic vsvs noncysticnoncysticdoes not evaluate does not evaluate extentextent

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

BranstetterBranstetter, 20069

11stst Branchial Cleft Cyst, Type IIBranchial Cleft Cyst, Type II

Type IType IEctodermalEctodermal duplication of duplication of EACEACNear external auditory Near external auditory canalcanalUsually inferior and Usually inferior and posterior to tragusposterior to tragus

Type IIType IIAssociated with Associated with submandibularsubmandibular glandgland

BranstetterBranstetter, 20069

Type 1 First Branchial Cleft CystType 1 First Branchial Cleft Cyst

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

BranstetterBranstetter, 20069

LymphangiomaLymphangioma

mass

auricle

parotid

Branchial CystBranchial Cyst

NoncalcifiedNoncalcifiedmassmass

CT shows lesion CT shows lesion under SCMunder SCM

Malik et al, 20026

22ndnd Brachial Cleft CystBrachial Cleft CystT2 MRIT2 MRI

Posterior to R Posterior to R submandibularsubmandibularglandgland

Thickened walls Thickened walls suggest prior suggest prior hemorrhage or hemorrhage or infectioninfection

Gujar and Mukherji 20045

Anterior to carotid bifurcationAnterior to carotid bifurcation

Schroeder et al, 20074

Under the anterior SCMUnder the anterior SCM

Schroeder et al, 20074

Exiting skin medial to lateral border of SCMExiting skin medial to lateral border of SCM

Schroeder et al, 20074

Left 2Left 2ndnd BA FistulaBA Fistula

Anterior to carotid bifurcationAnterior to carotid bifurcationUnder the anterior SCMUnder the anterior SCMExiting skin medial to lateral border of SCMExiting skin medial to lateral border of SCM

Schroeder et al, 20074

Moir. 20048

Moir. 20048

Moir. 20048

22ndnd BA cyst with sinus BA cyst with sinus tract extending into tract extending into the pharynx above the the pharynx above the carotid bifurcationcarotid bifurcation

Schroeder et al, 20074

Watch the hypoglossal!

PreauricularPreauricular SinusSinus

Not related to 1Not related to 1stst

branchial cleft branchial cleft anomaliesanomaliesActive infection Active infection during excision during excision increases chance of increases chance of recurrancerecurrance

Moir. 20048

ThyroglossalThyroglossal Duct CystDuct Cyst

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

epithelial lining epithelial lining composed of either composed of either squamoussquamous or respiratory or respiratory epitheliumepithelium

confirm normal thyroid confirm normal thyroid tissuetissue

Learning Radiology.com200711

ThyroglossalThyroglossal Duct CystDuct Cyst

CT w/ ContrastCT w/ Contrast

Embedded in the Embedded in the strap musclesstrap muscles

Extends deep to Extends deep to involve the preinvolve the pre--epiglotticepiglottic spacespace

Gujar and Mukherji 20045

Moir. 20048

Moir. 20048

DermoidDermoid CystsCysts

Ectoderm and mesodermEctoderm and mesoderm7% of 7% of dermoiddermoid cysts occur in head and cysts occur in head and neckneckThought to be of congenital inclusion Thought to be of congenital inclusion typetypemean diameter = 1.2 cm (0.6mean diameter = 1.2 cm (0.6--3.3)3.3)Treatment: complete excisionTreatment: complete excision

Pryor et al 200512

DermoidDermoid Cysts Cysts –– Cranial TheoryCranial Theory

GrunwaldGrunwald in 1910in 1910As As neuroectodermalneuroectodermal tract recedes, tract recedes, demaldemalattachementsattachements follow its course and can follow its course and can form a sinus or cystform a sinus or cystBeware of possible intracranial Beware of possible intracranial involvementinvolvement

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 sitesDiff Diff dxdx: in midline of neck: : in midline of neck: thyroglossalthyroglossal duct cystduct cyst

in head & neck, n = 59

Pryor et al 200512

DermoidDermoid CystsCysts

H & E

CT of dermoid cyst

Pryor et al 200512

TeratomaTeratoma

H&N account for ~2% of H&N account for ~2% of teratomasteratomasNewborn Newborn –– 2.5 yr at 2.5 yr at presentationpresentationAll 3 germinal layers presentAll 3 germinal layers presentMostly benign lesions Mostly benign lesions amenable to curative amenable to curative excisionexcision

WakhluWakhlu A et alA et al 200013

TeratomaTeratoma

•• Prognosis good if no Prognosis good if no respiratory compromiserespiratory compromise

•• Usually well Usually well differentiated and differentiated and recurrence is uncommonrecurrence is uncommon

•• Antenatal diagnosis is Antenatal diagnosis is routine in developed routine in developed worldworld

WakhluWakhlu A et alA et al 200013

TeratomaTeratoma

•• Proximity to vital Proximity to vital structures makes surgery structures makes surgery technically demanding.technically demanding.

•• Evaluate post op thyroid Evaluate post op thyroid and parathyroid function.and parathyroid function.

WakhluWakhlu A et alA et al 200013

TeratomaTeratoma –– 3 germ layers3 germ layers

Arise from Arise from pluripotentpluripotentcells and cells and ectopicectopicembryogenicembryogenicnonnon--germ germ cellscells

WakhluWakhlu A et alA et al 200013

TeratomaTeratoma –– 3 germ layers3 germ layers

WakhluWakhlu A et alA et al 200013

TeratomaTeratoma –– 3 germ layers3 germ layers

WakhluWakhlu A et alA et al 200013

HypopharyngealHypopharyngeal TeratomaTeratoma

calcified calcification and fat

Malik et al, 20026

TeratomaTeratomaT1 MRIT1 MRI

Fatty

Calcified

Gujar and Mukherji 20045

LymphangiomaLymphangioma

Benign, Benign, multiloculatedmultiloculated, soft, softPosterior neck triangle predominancePosterior neck triangle predominanceMultiMulti--septatedseptated, insinuating lesions, insinuating lesionsInfiltrate and cross tissue planesInfiltrate and cross tissue planesMost occur by 2 yrs of ageMost occur by 2 yrs of ageIncidence: 1 in 6,000 to 16,000 birthsIncidence: 1 in 6,000 to 16,000 births

BurezqBurezq 200614Head and and Neck Surgery,Head and and Neck Surgery, 200615

Lymphatic Vascular malformationLymphatic Vascular malformation

T1 MRIT1 MRI

High signal High signal represents represents proteinaceousproteinaceousfluidfluidCrosses tissue Crosses tissue planesplanes

Gujar and Mukherji 20045

Centrifugal Centrifugal vsvs CentripetalCentripetal

Centrifugal theoryCentrifugal theorythe lymphatic system develops as the lymphatic system develops as mesenchymalmesenchymalspaces that later coalesce into a system of spaces that later coalesce into a system of vessels that eventually join the venous system.vessels that eventually join the venous system.

Centripetal theoryCentripetal theoryjugular and posterior jugular and posterior lymphaticslymphatics form as form as outgrowths of endothelium from veins into the outgrowths of endothelium from veins into the surrounding surrounding mesenchymemesenchyme..

BurezqBurezq 200614

ClassificationClassification

Size:Size:MicrocysticMicrocystic: capillary : capillary lymphangiomaslymphangiomas

lesions are less than 1 cm in diameterlesions are less than 1 cm in diameter

MacrocysticMacrocystic: cystic : cystic hygromashygromascysts are larger than 1 cmcysts are larger than 1 cm

Cystic Cystic hygromashygromas #1 type of #1 type of lymphangiomalymphangioma

Gross et al, 200616

Cystic Cystic HygromaHygroma

NoncalcifiedNoncalcified

SeptatedSeptated on on U/SU/S

Malik et al, 20026

Cystic CompositionCystic Composition

55--yearyear--old boy with old boy with lymphangiomalymphangiomaL parotid & L parotid & parapharyngealparapharyngeal spacespacemixed macromixed macro--andmicrocysticandmicrocystic typetypeTreated by surgical Treated by surgical resectionresection

Gross et al, 200616

Type 1 First Branchial Cleft CystType 1 First Branchial Cleft Cyst

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

BranstetterBranstetter, 20069

LymphangiomaLymphangioma

mass

auricle

parotid

BurezqBurezq et al, 2006et al, 2006(expert opinion)(expert opinion)

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

Cystic Cystic hygromahygroma

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

microcysticmicrocystic, , macrocysticmacrocystic and mixed lymphatic lesionsand mixed lymphatic lesions

BurezqBurezq 200614

Management Management -- ControversialControversial

Spontaneous resolution?Spontaneous resolution?Formation of new lymphatic channels?Formation of new lymphatic channels?

Serial aspiration?Serial aspiration?

SclerosantSclerosant Agents?Agents?OKOK--432 (432 (lyophiliziedlyophilizied mixture of lowmixture of low--virulence group A virulence group A SterpSterppyogenspyogens

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

BurezqBurezq 200614

Success with Serial AspirationsSuccess with Serial Aspirations

BurezqBurezq 200614

Success with OKSuccess with OK--432432

Supraclavicular macrocysticlymphangioma

Gross et al, 200616

HemangiomaHemangioma

Less than 1/3 present at birthLess than 1/3 present at birthUsually seen in 1Usually seen in 1stst few months of life and few months of life and enlarge progressivelyenlarge progressively90% cases involutes spontaneously90% cases involutes spontaneouslySclerosingSclerosing agents controversialagents controversial

GlutGlut--11

erythrocyteerythrocyte--type glucose transportertype glucose transporter

found only in found only in microvascularmicrovascular endothelia of bloodendothelia of blood––tissue tissue barriers such as in the central nervous system, retina, barriers such as in the central nervous system, retina, placenta, placenta, ciliaryciliary muscle, and muscle, and endoneuriumendoneurium of peripheral of peripheral nervesnerves

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

Mo et al, 200617

MacArther, 200618

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

2. Inflammatory Lesions2. 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 When does cervical lymphadenopathylymphadenopathy require FNA? require FNA?

Benign reactive lymph node may persist Benign reactive lymph node may persist for weeks to monthsfor weeks to monthsLymphoma can present the same wayLymphoma can present the same way

Rapkiewicz et al 200721

To FNA or not to FNA? To FNA or not to FNA?

Reactive Reactive lymphadenopathylymphadenopathy the most likely the most likely etiology of pediatric neck massesetiology of pediatric neck masses

Diagnostic Diagnostic dilemadilema: a mass that does not : a mass that does not resolve after initial treatmentresolve after initial treatment

Rapkiewicz et al 200721

FNA ancillary studiesFNA ancillary studies

Gram stain, cultureGram stain, cultureAcid fast stainAcid fast stainImunocytochemistryImunocytochemistryCytogeneticsCytogenetics

Rapkiewicz et al 200721

Limitations to FNALimitations to FNA

A lesion may not be homogenousA lesion may not be homogenousFNA samples only part of the massFNA samples only part of the massMay miss the true lesionMay miss the true lesion

Unable to appreciate histological Unable to appreciate histological architecturearchitecture

Rapkiewicz et al 200721

Time to contemplate open biopsyTime to contemplate open biopsy

Enlarging massEnlarging massPoor response to medical treatmentPoor response to medical treatmentSuspicious clinical courseSuspicious clinical courseUnusual image findingsUnusual image findingsSystemic symptomsSystemic symptoms

Rapkiewicz et al 200721

Case Case –– F.R.F.R.

8 8 y/oy/o female, female, hxhx + PPD several yrs prior+ PPD several yrs priorPresents with R cervical Presents with R cervical adenopathyadenopathyFNA suggests FNA suggests granulomagranulomaRepeat FNA Repeat FNA --> same result> same resultAFB stain and cultures negativeAFB stain and cultures negativeClarithromycinClarithromycin and and ethambutolethambutol startedstarted

Rapkiewicz et al 200721

Case Case –– F.R.F.R.

AdenopathyAdenopathy and pain increasedand pain increasedThird FNA nonThird FNA non--diagnosticdiagnosticCT shows bulky homogenous CT shows bulky homogenous lymphadenopathylymphadenopathy of R upper spinal of R upper spinal accessory and upper jugular chains.accessory and upper jugular chains.Open biopsy displayed Open biopsy displayed Hodgkin's Hodgkin's lymphoma.lymphoma.

Rapkiewicz et al 200721

Reactive Reactive LymphadenopathyLymphadenopathy

33--yearyear--old childold childMultiple Multiple hypoechoichypoechoiclesionslesionsvariable shape and variable shape and sizessizesconsistent with consistent with reactive lymph reactive lymph nodesnodes

Malik et al, 20026

Enlarged Lymph NodeEnlarged Lymph Node

NonspecificNonspecific

Cause:Cause:cryptococcalcryptococcaladenitisadenitisYeastlikeYeastlikefungusfungus

Gujar and Mukherji 20045

Atypical Atypical mycobacteriamycobacteria: ex: : ex: cryptococcuscryptococcus

Saprobe in Saprobe in naturenatureworldwide worldwide distributiondistributionFound in soilFound in soilPortal of entry Portal of entry is lungis lung

Atypical Atypical mycobacteriamycobacteria: ex: : ex: cryptococcuscryptococcus

Associated w/Associated w/AIDSAIDSorgan organ transplantationtransplantationLymphoreticularLymphoreticulardiseasesdiseases

½½ pts lack pts lack apprarentapprarentpredisposing predisposing factorsfactors

BartonellaBartonella henselaehenselae

HypoechoicHypoechoic masses masses with irregular rim of with irregular rim of isoechoicisoechoic tissuetissue

Biopsy: Cat Scratch Biopsy: Cat Scratch DiseaseDisease

Malik et al, 20026

BartonellaBartonella henselaehenselae

Gram Gram –– coccobacilluscoccobacillus22-- 14 day incubation14 day incubationDxDx: requires prolonged : requires prolonged incubation (2 + weeks)incubation (2 + weeks)Rx: erythromycin 1Rx: erythromycin 1--4 m 4 m (unclear efficacy)(unclear efficacy)Normally benign courseNormally benign course

Malik et al, 20026

In heart valve

PeritonsillarPeritonsillar AbcessAbcess

Soft tissue density in Soft tissue density in submentalsubmental spacespace

Malik et al, 20026

Retropharyngeal Retropharyngeal AbscessAbscess

Widening of Widening of prevertebralprevertebral spacespace

Malik et al, 20026

Retropharyngeal Space AbscessRetropharyngeal Space Abscess

Gujar and Mukherji 20045

RetropharyngealPeritonsillar

Malik et al, 20026

SppurativeSppurative Cervical AdenitisCervical Adenitis

hypoechoic

Malik et al, 20026

Thyroid AbscessThyroid Abscess

Malik et al, 20026

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

3. Non3. Non--inflammatory Benign inflammatory Benign LesionsLesions

Inclusion cyst 13 3% Fibromatosis 9 2% Keloid 1

Inclusion CystInclusion Cyst

Acquired Acquired dermoiddermoid cystscystsresult from a part of the skin being traumatically result from a part of the skin being traumatically implanted in the deeper layers after implanted in the deeper layers after ectopicectopic formation formation of a of a dermal cyst lined with dermal cyst lined with squamoussquamousepitheliumepithelium..

Congenital inclusion Congenital inclusion dermoiddermoid cysts form along cysts form along the lines of embryologic fusion and contain both the lines of embryologic fusion and contain both dermal and epidermal derivatives.dermal and epidermal derivatives.

DermoidDermoid cysts of the head and neck are thought to be cysts of the head and neck are thought to be the congenital inclusion type. the congenital inclusion type.

Pryor et al 200512

Inclusion CystInclusion Cyst

many cysts originate from the many cysts originate from the infundibularinfundibularportion of the hair follicle, and the more portion of the hair follicle, and the more general term, general term, epidermoidepidermoid cystcyst, is favored, is favored

Becker et a, 200519

Epidermal Inclusion CystEpidermal Inclusion Cyst

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

Becker et al, 200519

TorticollisTorticollis

FibromatosisFibromatosis ColliColli

SCMSCMIsoechoicIsoechoic massmass

CT shows CT shows isodenseisodensemass R sidemass R sideNote normal SCM on Note normal SCM on L sideL side

Malik et al, 20026

FibromatosisFibromatosis ColliColli -- FNAFNA

PaucicellularPaucicellularspecimenspecimen

Bland spindle Bland spindle cell cytologycell cytology

r/or/o nodular nodular fascitisfascitis and and fibrosarcomafibrosarcoma

Rapkiewicz et al 200721

FibromatosisFibromatosis ColliColli

r/or/o nodular nodular fascitisfascitis and and fibrosarcomafibrosarcoma

Roy, 200720

Moir. 20042

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

4. Benign Neoplasms4. Benign Neoplasms

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

NeurofibromaNeurofibroma

solitary lesionsolitary lesionvsvs

part of the generalized syndrome of part of the generalized syndrome of neurofibromatosisneurofibromatosis

NFNF--1, aka von Recklinghausen disease1, aka von Recklinghausen diseaseNFNF--22

Believed to arise from Schwann cell but Believed to arise from Schwann cell but origin uncertainorigin uncertain

NeurofibromaNeurofibroma

solitary lesionsolitary lesionvsvs

part of the generalized syndrome of part of the generalized syndrome of neurofibromatosisneurofibromatosis

NFNF--1, aka von Recklinghausen disease1, aka von Recklinghausen diseaseNFNF--22

Believed to arise from Schwann cell but Believed to arise from Schwann cell but origin uncertainorigin uncertain

NeurofibromaNeurofibroma

T2 MRIT2 MRI

Central low T2 Central low T2 signal is signal is characteristic of characteristic of neurofibromasneurofibromas

Gujar and Mukherji 20045

LipoblastomaLipoblastoma

Rare benign Rare benign mesynchymalmesynchymaltumor of tumor of embryonalembryonal fatfat

May clinically and May clinically and radiologicallyradiologicallymimic a mimic a hemangiomahemangioma

Collections of Collections of lipoblastslipoblasts ––multivuolatedmultivuolated w/ round nucleiw/ round nuclei

FNA

LipoblastomaLipoblastoma

Resembles Resembles embryological adipose embryological adipose tissuetissue

Surgical specimen

LipomaLipoma LipoblastomaLipoblastoma

Neonatal Neonatal GoiterGoiter

CT shows large peripheral rim CT shows large peripheral rim enhancing, low attenuation enhancing, low attenuation massmass

1: 4000 live births1: 4000 live births

Female 2x = Male Female 2x = Male predominancepredominance

Delayed ossification at bone Delayed ossification at bone endsends

Malik et al, 20026

Rovet et al, 200310

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

Pediatric Neck MassesPediatric Neck Masses

1. Congenital lesions1. Congenital lesions2. Inflammatory lesions2. Inflammatory lesions3. Non3. Non--inflammatory benign lesionsinflammatory benign lesions4. Benign 4. Benign neoplasmsneoplasms5. Malignant 5. Malignant neoplasmsneoplasms

5. Malignant Neoplasms5. 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

LymphomaLymphoma

Third most common pediatric cancerThird most common pediatric cancerIncidence: 11Incidence: 11--20 per million children20 per million childrenGeographical variance Geographical variance –– 50 % of 50 % of childhood cancers in equatorial Africachildhood cancers in equatorial Africa

Due to high incidence of Due to high incidence of BurkittBurkitt’’ss lymphomalymphoma

Male predominance 2.5:1Male predominance 2.5:1

Beware the Beware the supraclavicularsupraclavicular mass!mass!

35% of patients 35% of patients with H&N with H&N lymphoma lymphoma present with a present with a supraclavicularsupraclavicularmassmass

35% of pts with 35% of pts with suprclavicularsuprclavicularmasses had masses had lymphomalymphoma

Turkington et al 200522 TorsiglieriTorsiglieri et al., 1988et al., 198822

NeuroblastomaNeuroblastoma

NoncontrastNoncontrast T1 T1 MRIMRI

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

Gujar and Mukherji 20045

RhabdomyosarcomaRhabdomyosarcoma -- CTCT

Ill definedIll defined

enhancing soft enhancing soft tissue densitytissue density

areas of necrosisareas of necrosis

Malik et al, 20026

RhabdomyosarcomaRhabdomyosarcomaof the Masticator Spaceof the Masticator Space

NonNon--contrast T1 contrast T1 –– intermediate intermediate signalsignal

T2 T2 –– increased signalincreased signal

Gujar and Mukherji 20045

TorsiglieriTorsiglieri et al., 1988et al., 198822

Inflammatory lesions

27%

Malignant neoplasms, 11%

Benign neoplasms3%

Non-infammatory benign lesions

5%

Congenital lesions54%

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 1Total 445TorsiglieriTorsiglieri et al., 1988et al., 198822

ConclusionsConclusions

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

Beware of tuberculosis, cat scratch Beware of tuberculosis, cat scratch disease, atypical infectionsdisease, atypical infectionsBeware of systemic symptomsBeware of systemic symptomsBeware the Beware the supraclavicularsupraclavicular massmassConsider FNA or biopsy in the mass that Consider FNA or biopsy in the mass that does not resolve with treatment.does not resolve with treatment.

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