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Vascular Lesions of the Head & Neck Deborah Shatzkes, MD Professor of Radiology & Otolaryngology Lenox Hill Hospital Northwell Health New York, NY ASHNR 2017 Lecture Outline • ClassificaFon and nomenclature • Highly simplified MRI-based differenFal algorithm • Imaging appearance of commonly encountered lesions “Hemangioma” Orbital VM (aka “cavernous hemangioma”) Intraosseous VM (aka “ossifying hemangioma”) Facial nerve VM (aka “ossifying hemangioma”) Cavernous malformaAon (aka “cavernous hemangioma”) Cavernous sinus VM (aka “cavernous hemangioma”) • “Hemangioma/Angioma”: Wastebasket terms used to describe any vascular lesion • 1982: Mulliken and Glowacki published seminal work on biological basis of vascular lesions (PRS 69: 412-422) • 1992: InternaFonal Society for the Study of Vascular Anomalies (ISSVA) formed. Most recent classificaFon published 2014 “Nomenclature has been the major obstacle to our understanding and management of vascular anomalies”* *Mulliken JA, in Mulliken & Young’s Vascular Anomalies, 2 nd Edition, 2013 Biology aʹ la Mulliken • Vascular tumors (most commonly infanFle hemangiomas) are true neoplasms and grow by endothelial hyperplasia • Vascular malformaAons have a quiescent endothelium and are considered localized defects of vascular morphogenesis (“badly formed vessels”) Immunohistochemical marker GLUT-1 accurately distinguishes ISSVA ClassificaAon of Vascular Anomalies (2014) Vascular Tumors Benign • InfanFle hemangioma • Congenital hemangioma (RICH, NICH) • Pyogenic granuloma Borderline • Kaposiform hemangioendothelioma • Kaposi sarcoma Malignant • Angiosarcoma Vascular MalformaAons Simple • Capillary malformaFon • Venous malformaFon • LymphaFc malformaFon • Arteriovenous malformaFon Combined • CVM, CLM, LVM, CLVM, AVM-LM, CM-AVM Associated with other anomalies • Sturge-Weber issva.org/classification

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Page 1: Vascular Lesions of the Head & Neck

VascularLesionsoftheHead&Neck

DeborahShatzkes,MDProfessorofRadiology&Otolaryngology

LenoxHillHospitalNorthwellHealthNewYork,NY

ASHNR 2017

LectureOutline• ClassificaFonandnomenclature• HighlysimplifiedMRI-baseddifferenFalalgorithm

•  Imagingappearanceofcommonlyencounteredlesions

“Hemangioma”

OrbitalVM(aka“cavernoushemangioma”)

IntraosseousVM(aka“ossifyinghemangioma”)

FacialnerveVM(aka“ossifyinghemangioma”)

CavernousmalformaAon(aka“cavernoushemangioma”)

CavernoussinusVM(aka“cavernoushemangioma”)

•  “Hemangioma/Angioma”:Wastebaskettermsusedtodescribeanyvascularlesion

•  1982:MullikenandGlowackipublishedseminalworkonbiologicalbasisofvascularlesions(PRS69:412-422)

•  1992:InternaFonalSocietyfortheStudyofVascularAnomalies(ISSVA)formed.MostrecentclassificaFonpublished2014

“Nomenclaturehasbeenthemajorobstacletoourunderstandingandmanagementof

vascularanomalies”*

*Mulliken JA, in Mulliken & Young’s Vascular Anomalies, 2nd Edition, 2013

BiologyaʹlaMulliken•  Vasculartumors(mostcommonlyinfanFlehemangiomas)aretrueneoplasmsandgrowbyendothelialhyperplasia

•  VascularmalformaAonshaveaquiescentendotheliumandareconsideredlocalizeddefectsofvascularmorphogenesis(“badlyformedvessels”)

Immunohistochemical marker GLUT-1 accurately distinguishes

ISSVAClassificaAonofVascularAnomalies(2014)

VascularTumorsBenign•  InfanFlehemangioma•  Congenitalhemangioma(RICH,NICH)•  PyogenicgranulomaBorderline•  Kaposiform

hemangioendothelioma•  KaposisarcomaMalignant•  Angiosarcoma

VascularMalformaAonsSimple•  CapillarymalformaFon•  VenousmalformaFon•  LymphaFcmalformaFon•  ArteriovenousmalformaFonCombined•  CVM,CLM,LVM,CLVM,AVM-LM,

CM-AVMAssociatedwithotheranomalies•  Sturge-Weber

issva.org/classification

Page 2: Vascular Lesions of the Head & Neck

NomenclatureOLD NEW

Capillaryhemangioma HemangiomaPortwinestain CapillarymalformaFon

StrawberryhemangiomaCavernoushemangioma

VenousmalformaFonOssifyinghemangioma

Lymphangioma LymphaFcmalformaFonCysFchygroma

ImagingofH&NVascularLesions

• MRIappearancereflectshistologiccomposiFon•  Hemangiomas=cellulartumors:avidenhancement,onlymoderate!T2SI

•  ThemostcommonlyimagedvascularmalformaFonsareVM,LM(lowflow)andAVM(highflow)

• MRIappearancereflectsvascularityandspecificvesselsubtype

(Really)SimplifiedDifferenAalAlgorithm

Hemangioma

AVM

VenousMalformaFon

LymphaFcMalformaFon

(+)CE (-)CE

↑T2SI

↑↑T2SI

I.Hemangiomas(vasculartumors)•  InfanFlehemangioma(common)•  Congenitalhemangioma(rare)•  Kaposiformhemangioendothelioma

II.VascularMalformaAons

•  Venous•  LymphaFc•  Arterial-Venous(AVM)•  Complex(e.g.LVM)

LowFlow

HighFlow

I.InfanAleHemangioma:Clinical

•  Commonbenignendothelialtumorofinfancy•  Notpresentatbirth•  Appearafewweekslaterwithrapidgrowth–proliferaAvephase(PhaseI)

•  Regressslowlybeginningatage2-5–involuAngphase(PhaseII)

•  Typicallycompletelyregressbylatechildhood-involutedphase(PhaseIII)

•  Glut-1posiAve

InfanAleHemangioma(PhaseI)

•  Hypercellularandhypervasculartumors• Largevessels• EctodermallocaFon

Hemangioma

AVM

VM

LM

↑T2SI

↑↑T2SI

(+)CE (-)CE

Page 3: Vascular Lesions of the Head & Neck

InfanAleHemangioma

•  IntermediateT1andT2signal• ProminentfeedinganddrainingvesselsreflecthypervascularityinPhaseI

ParoAdHemangioma

•  ParoFdderivedfromectoderm•  “Lightbulb”enhancingparoFdmassininfantvirtuallypathognomonic

InvoluAngHemangioma(PhaseII)

•  Decreaseintumorvolume• IncreaseinintersFcesandfagycontent

InvolutedHemangioma(PhaseIII)

• MinimallyenhancingfagysohFssuemass• ExcessfatandbonyovergrowthmayresultinpersistentcosmeFcdeformity

PHACESSyndrome

PosteriorfossaHemangiomaArterialCardiacEyeSternal

PHACES

Page 4: Vascular Lesions of the Head & Neck

CongenitalHemangioma•  Rare•  Presentandfullyformedatbirth•  GLUT-1negaAve•  RICH:RapidlyInvoluFngCong.Hemangioma•  NICH:NonInvoluFngCong.Hemangioma•  PICH:ParFallyInvoluFngCong.Hemangioma•  Clinicaldiagnosiswithoutspecificimagingfeatures

NICH

2years 7years

KaposiformHemangioendothelioma(KHE)

•  “Borderline”vasculartumor•  Ill-defined,invasive,subQfatstranding•  >50%associatedwithKassabach-Merrigsyndrome(“hemangioma”withthrombocytopenia)

I.Hemangiomas(vasculartumors)•  InfanFlehemangioma(common)•  Congenitalhemangioma(rare)•  Kaposiformhemangioendothelioma

II.VascularMalformaAons

•  Venous•  LymphaFc•  Arterial-Venous(AVM)•  Complex(e.g.LVM)

LowFlow

HighFlow

II.VascularMalformaAons-Clinical

•  Presentatbirth•  GLUT-1negaFve•  GrowinconjuncFonwithsomaFcgrowthinalinearfashion

• Willnotregressspontaneously•  Highflow(AVM)andlowflow(VM,LM)•  Complexvariantsdemonstratemixedimagingfeatures

VenousMalformaAon(VM)

• Malformedvenouschannelswithsluggishflow• BrightT2,avidenhancement• Phlebolithscommon

T1C+

STIR

Hemangioma

AVM

VM

LM

↑T2SI

↑↑T2SI

(+)CE (-)CE

Page 5: Vascular Lesions of the Head & Neck

LebCheekandLipMass

•  WelldemarcatedthoughinfiltraFve•  Clinicallyevidentlesionmayrepresent“Fpoftheiceberg”

VenousMalformaAon

Frequentlytrans-spaFal,withcommonmasFcatorspace(masseter)andpalatalinvolvement

M

VenousMalformaAon

• IniFalpatchycontrastenhancement“fillsin”onmoredelayedimaging• OrbitalVM,aka“cavernoushemangioma”ismostcommonorbitalmassinadults

early latepre

VenousMalformaAon

Phlebolithsresultfromsluggishvenousflowandarehighlyspecific

30y/owomannoAcedintermieentlebcheekfullness

VenousmalformaFon

IntraosseousVM(“OssifyingHemangioma”)

•  Facialskeleton,skull,temporalbone(“facialn.ossifyinghemangioma”)•  Internalbonespicules

Page 6: Vascular Lesions of the Head & Neck

LymphaAcMalformaAon(LM)•  Macrocystsormicrocysts• Well-definedorinfiltraFve•  Enhancementcapsule/septae•  “Soh”lesions•  Pronetointernalhemorrhage

T1C+

T2

Hemangioma

AVM

VM

LM

↑T2SI

↑↑T2SI

(+)CE (-)CE

LymphaAcMalformaAon

• MacrocysFcformsimagelikecysts• “CysFcHygroma”=macrocysFcLM• Commonattherootoftheneck

LymphaAcMalformaAon

•  MicrocysFcvariantsmoreinfiltraFve• Lessfluid=lowerT2signal• “Beard”distribuFoncommon

OrbitalLM

• OrbitiscommonlocaFon• SohlesionsmaybedeformedbyopFcnerve• Presentwhenhemorrhageleadstoproptosis(fluidlevelsinorbitalmass=LM)

LymphovenousMalformaAon(LVM)

T1 T2fatsatT1C+fatsat

•  FormofcombinedvascularmalformaFon•  SharesfeaturesofLMandVM

AVM• Largevessels• Nodiscretemass• Tissueedema• Bonylysisandovergrowth

Hemangioma

AVM

VM

LM

↑T2SI

↑↑T2SI

(+)CE (-)CE

Page 7: Vascular Lesions of the Head & Neck

FacialAVM

•  Ill-defined;nodiscretemass• Lookforflowvoidsandlargefeedinganddrainingvessels

NasalAVM

•  FindingsofFssueedema(T2hyperintensity,mildenhancement)maybesubtle•  Lookforflowvoids!

AVM

Bonyovergrowthand/orlyFcdestrucFon

Hemangioma•  Cellularneoplasm•  Triphasicgrowth•  LargevesselsinPhaseI

AVM•  Vascularflowvoids•  Nodiscretemass•  Bonylysisandovergrowth

VenousMalformaAon•  Phleboliths•  Dynamicenhancement

LymphaAcMalformaAon•  MacrocysFcandmicrocysFc•  Hemorrhagicfluidlevels

(+)Enhancement (-)Enhancement

↑T2SI

↑↑T2SI

VascularLesionsofH&N•  Reserveterm“hemangioma”forvasculartumorsofinfancy

•  Historyisimportant(presentatbirth?)•  EnhancementpagernisgoodfirststepindisFnguishinglesions

•  Phleboliths(VM),Flowvoids(AVM)andfluidlevels(LM)areimagingclues

Thank you! HEMANGIOMA


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