BrazilianJournalofOtorhinolaryngology2021;87(3):370---373
www.bjorl.org
Brazilian
Journal
of
OTORHINOLARYNGOLOGY
CASE
REPORT
A
rare
case
of
endolymphatic
sac
hemangioma
in
a
patient
alleged
to
have
Ménière’s
disease
Valentina
Longoni
a,c,∗,
Paola
Scagnelli
b,
Giancarlo
Tirelli
a,
Vittorio
Pietro
Achilli
caAziendaSanitariaUniversitariaGiulianoIsontina,HeadandNeckDepartment,ENTClinic,Trieste,Italy
bOspedaleMaggiore,RadiologyDepartment,Lodi,Italy
cOspedaleMaggiore,ENTDepartment,Lodi,Italy
Received14September2020;accepted20October2020 Availableonline22November2020
Introduction
Primary lesions of the endolymphaticsac (ELS) that arise fromtheendolymphaticsacorductareoftenmisdiagnosed. ELS tumorsarerare, typicallydestructive neoplasm of theposteriortemporalbone.
In 1984 during an endolymphatic sac decompression a tumorarisingfromendolymphaticsacwasdiscovered;later Heffner in 1989 describedELS tumorsaslow-grade papil-lary adenocarcinomas.1 Hemangiomas arebenign vascular
tumorsthathavetobeincludedinthedifferentialdiagnosis oflesionsinvolvingtheendolymphaticsac.2
Iftemporalbonehemangiomasareveryrare(Fiereketal. showed0.21%ofcasesin1430intra-temporaltumors3),the
endolymphaticsachemangiomasareevenmorerare.Only twocaseshave been described inthe literature,onein a patientaffectedbyVonHippleLindau(VHL)disease.2,4
∗Correspondingauthor.
E-mail:longonivalentina@gmail.com(V.Longoni).
PeerReviewundertheresponsibilityofAssociac¸ãoBrasileirade
OtorrinolaringologiaeCirurgiaCérvico-Facial.
Temporalbonehemangiomasmostfrequentlyinvolvethe internalauditorycanal(IAC)andtheregionofthegeniculate ganglion. Facialnervehemangioma represents 0.7%of all intratemporaltumors.5
Progressivesensorineuralhearinglossandpulsatile tinni-tusarethemainsymptomsincasesofIAClocalization;facial nervedysfunctionand/orfacialspasmsaremuchmore fre-quentincaseofgeniculateganglionregioninvolvement,but auditoryorvestibularsymptomsarenotexcluded.6
Temporalbonehemangiomascanencase cranialnerves andcanbefindeveninthemiddleandexternalear.Years canpassbeforediagnosis.
Imagingisnecessarytoexcludeotherpossibleetiologies ofapatient’ssymptoms.Intratemporalhemangiomasmimic othermorecommonskullbase lesions,whichmakesthem difficulttobediagnosedpreoperatively.
Hemangiomaoftheendolymphaticsaccouldbe misdiag-nosedwithavarietyofbothbenignandmalignantlesions, includingsecondarytumors,andinflammatoryconditions.
Case
report
A48-year-oldcaucasianwoman,previouslytreatedfor sev-eral years for Ménière’s disease with a low-sodium diet,
https://doi.org/10.1016/j.bjorl.2020.10.011
1808-8694/©2020Associac¸˜aoBrasileiradeOtorrinolaringologiaeCirurgiaC´ervico-Facial.PublishedbyElsevierEditoraLtda.Thisisanopen
BrazilianJournalofOtorhinolaryngology2021;87(3):370---373
Figure 1 High resolution CT scan ofthe temporalbone revealed a retrolabyrinthine boneerosion in theregion ofthe left endolymphaticsac,withirregularmarginsanda‘‘moth-eatenpattern’’(A).ThemasshadanisointensesignalonMRIT1-weighted images(B),showedamarkedcontrastenhancement(C),ahyperintenseheterogeneoussignalonT2-WI(D).
diuretics and betahistine without improvement, came to our clinic reporting a new episode of severe dizziness without vertigo. She also reported a history of pro-gressive unilateral left hearing loss without tinnitus. No headache, nausea, dysphagia, or otalgia were reported. She gave a history of head trauma in young age with-out developing of cranial nerve, including facial nerve ,palsies.
Onphysicalexaminationthetympanicmembraneswere bilaterally intact. There was no evidence of spontaneous or evoked nystagmus; during a Romberg test multidirec-tionaloscillopsiesweredetected.NodisfunctionofVII,IX, X,XI,XIIcranialnerveswereobserved.Leftmildfacialpalsy (House-BrackmanngradeII)wasobserved.Toneaudiometry revealed asymmetricsensorineural hearingloss,severeon theleftear.
Brainstemauditoryevokedresponsesshowedonlyslightly increasedlatencyontheleftsideforwaveV(ILD-V0,28).
The caloric test showed asymmetrical vestibular responseswithleftsidehyporeflexia.
Thehigh-resolutioncomputedtomography(HRCT) with-out contrast enhancement, with bone algorithm scan, showed a bone erosion in the region of the left ELS. A brain and temporal bone magnetic resonance imaging (MRI) with gadolinium-DTPA (diethylenetriamine penta-acetic acid) revealed an osteolitic lesion in the posterior face of the left petrous bone, in the region of the ELS, respectively isointense to brain on T1-weighted images, hyperintenseonT2-weightedenhancedimages,andmarked contrastenhancementimages(Fig.1).
Intraoperative monitoring of VII leftcranial nerve was conducted.
Atransmastoidretrolabyrinthineapproachundergeneral anesthesia, wasperformed to gain access,preservingthe oticcapsule. An extended posteriorleft auricularincision wasmade.Adeepmastoidectomywasnecessaryto skele-tonizethesigmoidsinusandthejugularbulb.Theposterior semicircularcanal wasidentifiedandpreserved.The mas-toidsegmentof thefacialnervewasskeletonizedbutnot exposed(Fig.2).
Theposteriorfossadurawasexposed,removingthebone betweenthesigmoidsinusandthebonylabyrinth.TheELS andthevestibularaqueductwereidentifiedinferiorto Don-aldson’sline.
An osteolytic lesion of the ELS, infiltrating underlying posterior fossa dura became apparent and was radically removed.Theposteriorlabyrinthwasundamaged.
The posterior fossa duraopening wasrepaired withan abdominalfatplug.
Histopatologicalexaminationrevealedahemangiomaof theELS(Fig.3).
The postoperative course was uneventful: hearing unchanged,dizzinessimprovedbutpersistent,andvertigo didnotoccur.
Thepatienthasnotrecurrencesduring12months follow-up.
Discussion
V.Longoni,P.Scagnelli,G.Tirellietal.
Figure2 Leftretro-auricular transmastoidapproach withpreservation oftheposterior meatalwall,andskeletonizationthe sigmoidsinus(A);skeletonizationoftheposteriorsemicircularcanalandremovaloftheretrofacialcellsinordertoexposetheregion oftheEndolymphaticSac(ELS)(B);aretrolabyrinthineboneofredappearancewashighlightedbydrillingbelowtheDonaldson’s line.ItlookedinfiltratedanderodedbyareddishvascularizatedmassenvelopingtheELS(C);theELSwithadjacentposteriorfossa durawereremovedbecauseinvolvedinthelesion(D).
endolymphaticsystemsecondarytoELShemangioma,orby asortof‘‘vascularstealeffect’’duetothehighly vascular-izedlesion.Also,theaudiogramcouldpresent acombined low and high frequency hearing loss because of degener-ation of the organ of Corti due to the distention of the membranouslabyrinth.6
CrosssectionalimagingasMRIandHRCTmayberequired notonlytoexcludeacousticneuroma,meningiomas, para-gangliomas,ELTsorothercerebellarpontineanglelesions, butalsointracranialpathologiescausingbalanceand hear-ingdisorderssuchasmultiplesclerosisorhydrocephalus.
TherearefewreportedcasesofELTsoccurringinpatients with Ménière’s disease; both cases of ELS hemangiomas reported have atypical Ménière’s triad. The first one had historyofvertigoandunilateraltinnituswithnormal hear-ingbilaterally,2 whiletheVHL patientpresentedasudden
sensorineuralhearingloss.4
InVonHippel---Lindaudiseasefrom11%to16%ofpatients develop ELTs that occurbilaterally inabout 30% of cases. The association between VHL and these tumorshas been identified and confirmed by few authors. ELS tumors and hemangiomata could mimic Ménière’s disease present-ing initially unremarkable imaging; these images are not clearly radiologically distinguishable from temporal bone ELStumorsorvascularentity,consideringthatvascular mal-formation maycoexistina singlemass.ELS hemangiomas areusuallyrevealedbyaretrolabrynthineboneerosionwith irregularmarginsusuallydescribedasa‘‘moth-eaten pat-tern’’; intratumoral bone spicules present nearly all the timein lesionscentered overthe endolymphaticsac, and may extendinto the IAC,the medial portionof the mas-toid to invade facial nerve or into the posterior cranial fossa to invade the dura on HRCT exam. A honey-comb
signonHRCTcouldbepresentandcorrelate withtheMRI nonhomogeneoussignalintensity.OnMRIthemasshas usu-ally an isointense signal on MRI T1-weighted images, a hyperintenseheterogeneoussignalonT2-weighted images andshowsmostlyinhomogeneouscontrastenhancement.7,8
Hemangiomasof thetemporalbone couldpresent adural tailsign,andcouldbeconfusedwithmeningiomas preoper-atively.Furtherclassificationofhemangiomasisproposedin literature;MullikenandGlowackiproposedaclassification systemfor vascularlesions.9 Thedifferentialdiagnosis for
hemangiomaincludesbenignandmalignanttemporalbone neoplasms. Paragangliomas are by far the most common. The temporal bone could be a localization of metastatic papillary thyroid carcinoma, or renal carcinoma. Facial nerveschwannomas,choroidplexuspapillomas,ceruminous neoplasms, and meningiomas couldbe misdiagnosed with hemangioma.Myelomas, nasopharyngeal carcinomas, xan-thomas,petrous apexabscessesor osteomyelitismay also beincludedasdifferentialdiagnosis.Thereis avarietyof namesemployedforELStumors;particularlyendolymphatic sac papillary tumor has a lot of synonyms, this reflects the histogenetic and behavioral uncertainties. They have benign but invasive nature, they are locally aggressive, anddistantmetastasis is described butexceptionallyrare even if possible. Clinical and radiological findings cannot alwaysdifferentiatehemangiomafromother,neoplastic,or inflammatorydisease,gadolinium-enhancinglesionsofthe ELS,1sosurgicalresectionremainsthetreatmentofchoice.
BrazilianJournalofOtorhinolaryngology2021;87(3):370---373
Figure3 (EE10×)Low-powermicroscopicviewofthe spec-imenshowingirregulardilatedvascularspaces,devoidofred bloodcells,infiltratingbonetrabeculaeandfibroustissue(A); (EE40×)athighpowerthevascularspacesarelinedbyasingle rowofendothelialcellwithoutunderlyingmuscularwall(B). sacrificingresidualhearingisabetterchoice.Ifthelesion involvestheposteriorlabyrinth,labyrinthectomyhastobe performed.2 Atranscanal endoscopicapproach, combined
witharetroauriculartransmastoidminicraniotomy,hasbeen describedinacaseoffacialnervehemangiomainorderto respectthe labyrinthineblock andcochlea.5 Nevertheless
itisnotexactlyclearifhemangiomasaretobeconsidered vasculartumorsormalformations.10
Conclusions
Hemangiomas, depending onlocalizationand clinical pre-sentationcanbemistakenforamyriadoftypesoflesions. HRCT scan without contrast enhancement and contrast-enhanced MRI of the temporal bone and skull base are recommendedforpatientsinwhomMénièrediseaseis
sus-pected,butthe‘‘symptomstriad’’isatypicalornotpresent. Imaging surveillance protocol in patient’s suspected for Ménière disease could be useful to control new onset of ELStumor,theadequateregularityisnotclearpredictable. Sinceradiologicalevaluationcouldnotbeenoughtomakea preoperativediagnosis,thesurgicalresectionisthechoice toprovidea histopathologicaldefinition. Because oftheir locally invasive nature, waiting and scanning cannot be recommended.A transmastoid retrolabyrinthine approach allowsthesurgeontopreserveoticcapsule.Radical resec-tionisrecommendedincludingtheduramatercoatingthe sac,in order toachieve a complete removaland prevent recurrence.Surveillance strategies have tobe considered worthwhileinpatientswithVHL.
Conflict
of
interest
Theauthorsdeclarenoconflictsofinterest.
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