jcoloproctol(rioj).2017;37(3):247–250
w w w . j c o l . o r g . b r
Journal
of
Coloproctology
Case
Report
Hemipelvectomy
with
laparoscopic
abdominoperineal
excision
for
epithelioid
sarcoma
treatment
夽
Daniel
Paulino
Santana
a,
Matheus
Matta
Machado
Mafra
Duque
Estrada
Meyer
a,∗,
Edmilson
Celso
Santos
a,
Camila
Gomes
de
Souza
Andrade
b,
Paulo
Vilela
Neto
b,
Aline
Alves
Matoso
c,
Bruna
Sílvia
Torres
Santos
c,
Bruno
José
Guedes
Silva
c,
Danielle
Bossi
Grassi
Ferreira
c,
Larissa
Vasconcelos
Horta
c,
Marcos
Campos
Wanderley
Reis
daHospitaldaBaleia,BeloHorizonte,MG,Brazil
bHospitaldaBaleia,ProgramadeResidênciaMédicadeCirurgiaGeral,BeloHorizonte,MG,Brazil
cFaculdadedeMedicinadeBarbacena,Barbacena,MG,Brazil
dHospitaldaBaleia,Servic¸odeCirurgiaGeral,BeloHorizonte,MG,Brazil
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Articlehistory:
Received28August2016 Accepted27March2017 Availableonline12May2017
Keywords:
Sarcoma
Epithelioidsarcoma Softtissuesarcoma Neoplasm
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Theepithelioidsarcoma(ES)isanunusualvariantofthesarcoma,notreaching1%ofall softtissuesarcomas.Initiallyreportedin1970,ithasamoreaggressivevariant,whichwas classifiedasthe“proximal-type”in1997.Theseareaggressivetumorswithhighratesof distantmetastasisandlocalrelapses.Isolatedradioandchemotherapyresponsesarepoor andfree-marginsurgicalresectionisthetreatmentofchoice.
Thisisthecasereportofa25-year-oldmalepatientdiagnosedwith“proximal-type”ES in theperinealregion.Heunderwent surgicalresectioninanother institutionandwas later admitted toour institutionwith localrecurrence withrectal andleft thigh mus-cleinvasion.Neoadjuvantradio-chemotherapywasperformed,followedbylaparoscopic abdominoperinealrectal resectionandpartial lefthemipelvectomyassociatedwith left lower-limbamputation.Thepatienthadnopostoperativecomplicationsandiscurrently undergoingoutpatientfollow-up.The anatomopathologicalanalysisshowedtumor-free margins.
The“proximal-type”ESisararesoft-tissuesarcomasubtype.Highlocalrecurrence,as wellasthemetastasisrates,makesitstreatmentachallengingtask.
©2017SociedadeBrasileiradeColoproctologia.PublishedbyElsevierEditoraLtda.This isanopenaccessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/ licenses/by-nc-nd/4.0/).
夽
StudyconductedatFundac¸ãoBenjaminGuimarães,HospitaldaBaleia,DepartamentodeCirurgiaGeral,BeloHorizonte,MG,Brazil.
∗ Correspondingauthor.
E-mail:matheusww@gmail.com(M.M.Meyer).
http://dx.doi.org/10.1016/j.jcol.2017.03.005
248
jcoloproctol(rioj).2017;37(3):247–250Hemipelvectomia
com
amputac¸ão
laparoscópica
do
reto
para
tratamento
de
sarcoma
epitelioide
Palavraschave:
Sarcoma
Sarcomaepitelióide Sarcomadetecidosmoles Neoplasia
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Osarcomaepitelióide(SE)éumsubtipoincomumdesarcoma,nãochegandoa1%dos sar-comasdepartesmoles.Foiinicialmentedescritoem1970,sendoqueem1997umavariante maisagressivafoiclassificadacomo“tipoproximal”.Sãotumoresagressivos,comaltas taxasdemetástaseserecidivalocal,comrespostapobreàquimioterapiaeradioterapia isoladas.Aressecc¸ãocirúrgicacommargenslivreséotratamentopadrão.
Apresentamos ocaso de um paciente de 25 anos com SE tipoproximal avanc¸ado em região perineal, submetidoa ressecc¸ão em outro servic¸o, evoluindo com recidiva local cominvasão docanalanal emusculaturada coxa esquerda.Foirealizado trata-mentoradioquimioterápiconeoadjuvanteseguidoderessecc¸ãocirúrgicacomamputac¸ão abdominoperineal do reto por videolaparoscopia associadaa hemipelvectomia parcial esquerdaincluindoomembroinferioresquerdo(MIE).Opacienteapresentouboaevoluc¸ão pósoperatóriaeencontra-seemsegmentoambulatorial.Oanatomopatológicoevidenciou margenscirúrgicaslivres.
OSEproximaléumtiporarodesarcomadepartesmoles,cujotratamentocurativoé desafiador,emvirtudedosaltosíndicesderecidivalocalemetástasesàdistância.
©2017SociedadeBrasileiradeColoproctologia.PublicadoporElsevierEditoraLtda.Este ´eumartigoOpenAccesssobumalicenc¸aCCBY-NC-ND(http://creativecommons.org/ licenses/by-nc-nd/4.0/).
Introduction
Soft tissue sarcomas are rare neoplasms, accounting for approximately 1% of malignant tumors in adults. These tumors originatefrom the mesenchyme, which can result inseveralhistologicalsubtypes.Theepithelioidsarcoma(ES) consistsinararesubtype,correspondingtolessthan1%of soft-tissuesarcomas.ItwasfirstdescribedbyEzingerin1970,1
withatypicalhistologyofgranulomatoustissue, character-izedbythepresenceofmultiplenodules,spindle cellsand centralnecrosis.Theyoccurmoreofteninthedistal topog-raphyofyoungadultlimbs,especiallyinthehands.Amore aggressivevariant wasdescribedbyGuillou in1997,2 being
called“proximaltype”,withgreateroccurrenceinthepelvis, perineum,andgenital tract,alsoinyoungindividuals.The typicalgranuloma-likehistologyoftheconventionalESwas showntobelessfrequent,addedtothepresenceof polygo-nalcellswithepithelioidandrhabdoidtraits,similartothe findingsofundifferentiatedcarcinomas.
The“proximaltype”ESisatumorwithaworse progno-siswhen compared toconventional ES, with high rates of localrecurrence,aswell ashematogenous metastasesthat can vary between 30 and 50% ofthe cases.3 Lymph node
metastasisisunusualinsarcomas, beingmorecommon in cases of proximal ES. These are tumors that show little responsetochemotherapyandradiotherapyalone,withthe standardtreatment beingsurgical resectionwithfree mar-gins.Tumorsizeseemstobetheonlywell-definedprognostic factor.
OverallsurvivalforpatientswithconventionalESvaries from 50 to70% in5years,3–6 whereas the casesof
“proxi-maltype”SEhavelowersurvival,with65%ofcasesrecurring and75%havingmetastases,especiallyinthelymphnodes.7
Approximately65%ofpatientsdieofthedisease,mostofthem duetometastaticdisease.7
Theaimofthisstudywastoreportacaseofadvanced prox-imalepithelioidsarcomaintheperinealregionina25-year-old patientundergoingmultidisciplinarytreatmentinour institu-tion.
Case
report
A 25-year-old male patient, with a hard and ulcerated palpable mass in the left perianal region, with an initial diagnosis ofperianal abscess, admitted toanother service where an unsuccessful drainage attempt was performed, followedbysurgicalresection.Anatomopathological exami-nationrevealeda3.5×3.2cmlesionwithepithelioidsarcoma microscopyresultwithfreesurgicalmargins.
jcoloproctol(rioj).2017;37(3):247–250
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Fig.1–In(A)ulceratedtumorlesionintheperinealregionextendingtotheleftthigh;(B)inthedetail,ulceratedlesion,and perinealbleeding.
Thepatientshowedclinicalprogressionofthelesion,with incapacitatingpainintheleft lowerlimb,permanent semi-flexedposition,andskinulcerationintheleftperianalregion with continuous small bleeding. The re-staging with MRI showedmassiveheterogeneousexpansiveformationinthe leftperinealregion,solid,withareasofnecrosisinitsinterior, involvingthedeepmuscularplanes,withnocleavageplanes withtheanalcanalontheleft,andnosignsofinvasionofthe genitourinarytract.Itextendedthroughthemusculatureof themedialaspectoftheleftthightoitsdistalthird,measuring about22.0×9.5×6.0cm.Inguinallymphadenomegalieswere identifiedontheleft.Thepatientwasthenadmittedforpain controlandpreparedforsurgerybyamultidisciplinaryteam (OncologicSurgery,Coloproctology,Urology,Orthopedics,and PlasticSurgery)withaproposalofabdomino-perineal ampu-tationoftherectusthroughvideolaparoscopy,associatedwith lefthemipelvectomyincludingtheLLL(Fig.1).
Initially,aminimallyinvasivevideolaparoscopicapproach wasusedtodissectthe entirerectosigmoid,withcomplete excisionofthemesorectumnearthepelvicfloor,ligatureof theinferiormesentericvessels,andligationoftheleft inter-nal iliac artery, aiming to reduce bleeding in the perineal approach.Aterminalcolostomywascreatedontheleftflank. Intheperinealarea,therectumwasamputatedtogetherwith softpartsoftheperineumontheleft,withfreemacroscopic margins, preserving the genitourinary tract,which did not showanyinvasion.Leftinguinal dissectionwasperformed, associatedwithleftpartialhemipelvectomytogetherwiththe LLL(Fig.2).Thereconstructionwascarriedoutusinga bio-logicalmeshofporcineacellulardermaltissue(Permacol®) forcorrectionofthepelvicdefect,aswellascoverageusing alateralmusculocutaneousflapofthethigh,astherewasno evidenceoftumorinvolvement(Fig.3).Thesurgicalprocedure wasuneventful,withatotaldurationof7h,with600mLof packedredbloodcellbeingtransfusedduetothepreviously lowlevelsofhemoglobin.ThepatientwasreferredtotheICU, hemodynamicallystable.Heshowedafavorablepostoperative evolution,beingdischargedfromthehospitalafter9days.
Anatomopathological examination disclosed a poorly differentiated proximal epithelioid sarcoma measuring 38×11×6cm, with 60% necrosis, affecting soft tissues in thedeepplanesoftheperinealregionandthemedialthigh area,reachingthepoplitealfossa.Therectosigmoidsegment showedmultifocalinfiltration.Angiolymphaticand perineu-ral invasion were present, with free proximal, distal, and radialmargins.Eleveninguinaland8peri-rectallymphnodes weredissectedwithoutneoplasticinvolvement.
Thepatientisbeingfollowedupinanoutpatientsetting, withnosignsoftumorrecurrence.
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jcoloproctol(rioj).2017;37(3):247–250Fig.3–Finalaspectafterreconstructionwithlateralthigh flap.
Discussion
The proximal ES is a rare subtype of soft tissue sarcoma corresponding to less than 1% of all types, with a higher incidenceinyoungmaleadults.Thediagnosisconsidersthe histopathologicaland immunohistochemicalcharacteristics forepithelialmarkers.Theproximalsubtypediffersfromthe classictypeduetoitsmultinodulargrowthpattern, predom-inantlyproximallocation(pelvis,perineumandgenitaltract) andmoreaggressiveclinicalbehaviorsincethebeginningof the presentation,8 withhigh ratesoflocalrecurrence after
resection, and hematogenous metastasis, especiallyto the lungs,andlymphaticareas.
ThereisnoconsensusaboutthetreatmentofproximalES. Mostpatientsundergoamultimodaltreatmentthatincludes surgicalresection,plusradiotherapyandchemotherapy. Stud-ieswithneoadjuvantchemotherapyregimensforsofttissue sarcomasindicatea30–60%responserateandasignificant improvement in recurrence and disease-free survival. The associationwithradiotherapyseemstoincreaseresectability andlimbpreservationrates.Additionally,adjuvant radiothe-rapyhasawell-definedroleinthebestlocalcontrolofthe disease,beingindicatedforhigh-gradetumors,thoselarger than5cmand/orcompromisedsurgicalmargins.9
Inthe present case,combined chemotherapy with ifos-famide and doxorubicin,associated withradiotherapy was carriedoutasneoadjuvanttherapyduetothelargeextension
of the lesion, aiming to increase the chances of curative resection.Themultidisciplinaryapproachconsistedof exten-siveresectionwithenlargedlaparoscopicabdominoperineal amputation to the left perineal region associated with en bloclefthemipelvectomy.Theanatomopathological examina-tion showedfreesurgicalmarginsandtherewasnolymph nodeinvolvementamongthe19dissectedlymphnodes,even though it was aproximal epithelioidsarcoma, wheresuch occurrence is more frequent. These patients require strict postoperativefollow-uptodetectlocal,regionalanddistant metastases.
Conclusion
TheproximalESisararetypeofsofttissuesarcoma,ofwhich histological diagnosis is difficult, as well as its treatment, duetohighratesoflocalrecurrence,distantmetastasisand lowresponsetochemotherapyandisolatedradiotherapy.In manycases,extensivesurgicalresectionwithgreat mutilat-ingpotentialarenecessary,inviewoftheirproximalsiteof involvement.Postoperativefollow-upshouldbestringent,and theprognosisispoor,since80%ofcasesdiefromthedisease withinthefirst3yearsaftertheinitialdiagnosis.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
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