Pleasecitethisarticleinpressas:BarhmiI,etal.Angiosarcomaoftheauricle.EuropeanAnnalsofOtorhinolaryngology,HeadandNeck diseases(2016),http://dx.doi.org/10.1016/j.anorl.2016.03.005
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Case report
Angiosarcoma of the auricle
I. Barhmi
∗, R. Abada , M. Roubal , M. Mahtar
Serviced’ORLetchirurgiecervico-faciale,Hôpital20Août1953,CHUIbnRochd,Casablanca,Morocco
a r t i c l e i n f o
Keywords:
Angiosarcoma Auricle Treatment
a b s t r a c t
Introduction:Angiosarcomasarerarebutaggressivevascularcancersofendothelialcellorigin.Thediag- nosisisoftendifficult,basedonspecificimmunohistologicalfeatures.Theycharacteristicallypresenta highlocalrecurrencerateandanearlymetastaticpotential.
Casereport:Wereportacaseofangiosarcomaoftheauricleina31-year-oldfemalepatientwhopresented withswellingoftheleftearfor3years.Histologicalexaminationdemonstratedangiosarcoma.Treatment consistedofsurgeryfollowedbypostoperativeradiotherapy.
Discussion: Thiscasereportdescribestheepidemiologicalandclinicalprofileandthetreatmentand outcomeofangiosarcomaoftheauricle,togetherwithareviewoftheliterature,demonstratingcertain diagnosticandtherapeuticdifficultiesinthemanagementofthisaggressivetumour.
©2016PublishedbyElsevierMassonSAS.
1. Introduction
Sarcomasaremalignantmesenchymaltumours,representing lessthan1%ofallcancers[1].Angiosarcomasrepresent2%ofall sarcomasandarisefromvascularendothelialcells[2].However, 50%ofallangiosarcomasinvolvetheskinoftheheadandneck [3].Thediagnosisofthistumourisoftendelayedbyitsapparently benignclinicalpresentation[4],whichcanbeconfusedwithaskin infectionorsofttissuetrauma[4].Theauricleisanexceptional siteofangiosarcoma,as,toourknowledge,only4caseshavebeen reportedintheliterature[5].
2. Casereport
A31-year-oldwoman,withnonotablehistory,presentedwith progressiveswellingoftheleftearfor1yearinacontextofgood generalhealth. Askininfection wasinitiallysuspected andthe patientwastreated withantibioticsfor three months,andwas subsequentlylosttofollow-upfor9months.Clinicalexamination revealedapainlesssphericallesioninthepinnaoftheleftearmea- suring4cm×4cm,thatbledonpalpation,withnofacialpalsyor cervicallymphnodesandwithnormalotoscopy(Fig.1).
Computed tomography showed a soft tissue tumour invad- ingtheleftauricleandadjacentskin(Fig.2),withnounderlying contrast-enhancedbonelesion.
∗ Correspondingauthor.
E-mailaddress:ismailbarhmi@gmail.com(I.Barhmi).
Biopsyofthelesionandtheleftpre-auricularskinrevealeda diagnosisof angiosarcoma. Immunohistochemistryrevealed the following results: vimentin (+), cytokeratin (−) and S-100 (−).
Treatmentconsisted ofcomplete surgicalresectionofthemass withsuperficialparotidectomyand systematiclevelsI,IIandIII lymph nodedissection(Fig.3).Histologicalexamination of the operativespecimendemonstratedmoderatelywelldifferentiated high-gradeangiosarcomainvadingmuscleandcartilagewithnega- tivelymphnodesN0R−.Externalbeamradiotherapywasdelivered tothetumourbedatadoseof50Gy.After6monthsoffollow-up, thispatientwasingoodgeneralhealthwithnosignsofrecurrence.
3. Discussion
Angiosarcomaisoneoftherarestsofttissuetumours;itisvery aggressiveandhasapoorprognosis[1].Itisaverywelldifferen- tiatedtumourthatcanbeconfusedwithanaplastictumoursand haemangiomas,whicharedifficulttodistinguishfromcarcinomas [3].Angiosarcomaoftheauricleisextremelyrare,as,toourknowl- edge,only4caseshavebeenreportedintheliterature[1–5].Itcan beconsideredtobeanangiosarcomaofthefaceandscalp[3].It hasapoorprognosis,witha5-yearsurvivalrateof12%[6].Theini- tialclinicalpresentationofthetumourisextremelyvariable.The patientoftenpresentslate,astheswellingisrarelypainful[3],and maysimplyresembleabruiseoranulceratedandinfectednodular tumour.Atamoreadvancedstage,thetumouristypicallyhae- morrhagicandulcerated[7]andmayresembleabenignsofttissue tumoursuchashaemangiomaormalignantskincarcinoma[4].This initialclinicalconfusioncanleadtodelayeddiagnosisandmaypre- disposetotumourprogression,whiletumoursizeconstitutesan http://dx.doi.org/10.1016/j.anorl.2016.03.005
1879-7296/©2016PublishedbyElsevierMassonSAS.
Pleasecitethisarticleinpressas:BarhmiI,etal.Angiosarcomaoftheauricle.EuropeanAnnalsofOtorhinolaryngology,HeadandNeck diseases(2016),http://dx.doi.org/10.1016/j.anorl.2016.03.005
ARTICLE IN PRESS
G Model
ANORL-531; No.ofPages2
2 I.Barhmietal./EuropeanAnnalsofOtorhinolaryngology,HeadandNeckdiseasesxxx(2016)xxx–xxx
Fig.1. Swellingoftheauricleoftheleftear.
Fig.2. CTscanshowinginvasionofthesofttissuesoftheleftauricle.
Fig.3. ResectionofthemasswithsuperficialparotidectomyandlevelsI,IIandIII lymphnodedissection.
importantprognosticfactor.IntheseriesreportedbyLydiattetal., allpatientswithanangiosarcomalargerthan10cmdied,while smallerlesionsmeasuring5cmwereassociatedwithahighersur- vivalrate,hencethevalueofearlydiagnosis[8].Onlyhistological examinationcanconfirmthediagnosis[5].Histologically,angiosar- coma is composed of a network of vascular anastomoses or a disorganizedsinusoidalappearancedissectingthedermalcollagen [7].Computedtomographyisusefultoevaluatethetumourarchi- tectureandcharacteristicsandforprecisestagingofthetumour[5]
withinvasionofthesofttissuesoftheearandtheadjacentskin[9].
Intheabsenceofconsensus,thePittsburghTNMclassification ofsquamouscellcarcinomasoftheexternalauditorycanal[9,10], proposed in 1990, is the most commonly used classification, based onthe findings ofpreoperative clinical examination and CTscan[8].Theadvantageofthisclassificationisthatitcanbe usedtoguidebothtreatmentandprognosis.Aminorrevisionwas proposedin2000[8].
Distantmetastasesmayinvolvecervicallymphnodes,aswellas liver,spleen,bone,kidneysandmyocardium[6].Surgicalresection withlargeanddeepresectionmarginsisrecommended[7].Inthe casereportedhere,andaftermultidisciplinaryconsultation,itwas decidedtoperformlevelsI,IIandIIlymphnodedissectioninaddi- tiontolargesurgicalresection,inviewofthehighincidenceof lymphnodemetastases(10–15%)[6].
The role of adjuvant radiotherapy remains controversial, as nostudy had demonstrated anybenefit on survival [3]. In the presenceofcervicallymphnodemetastases,Hodgkinsonetal.rec- ommendedlymphnodedissectionandradiotherapytothetumour bedandtotheneck[10].
Thesetumourshave a poorprognosisduetotheirtendency tolocalrecurrenceandlymphaticorhaematogenousmetastases.
Lydiattetal.reporteda5-yearsurvivalrateof33%,witharecur- rencerateof 78% [8].Our patient didnotpresent anysigns of recurrence6monthsaftertheendoftreatment.
4. Conclusion
Angiosarcomaoftheauricleisararetumour.Thediagnosisis delayedduetoitsnonspecificearlysigns.Histologicalexamination ofabiopsy,performedinthecaseofanysuspiciouslesions,isthe keytothediagnosisandtheonlywaytoensureeffectivetreat- ment.Multidisciplinarymanagementbyasurgeon,radiologistand radiotherapistisessential.
Disclosureofinterest
Theauthorsdeclarethattheyhavenocompetinginterest.
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