A case report - iKnow

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CASE REPORT – OPEN ACCESS

InternationalJournalofSurgeryCaseReports72(2020)91–95

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International Journal of Surgery Case Reports

j o u r n al ho m e p a g e :w w w . c a s e r e p o r t s . c o m

Well-differentiated liposarcoma disguised as a recurrent lipoma of the forearm ﬂexor compartment: A case report

Wahyu Widodo, Wildan Latief, Dina Aprilya

^∗

DepartmentofOrthopaedicandTraumatology,FacultyofMedicineUniversitasIndonesia,CiptoMangunkusumoHospital,Indonesia

a r t i c l e i n f o

Articlehistory:

Received30April2020

Receivedinrevisedform21May2020 Accepted21May2020

Availableonline30May2020

Keywords:

Giantlipoma Forearm

Welldifferentiatedliposarcoma Lipoma-like

a b s t r a c t

INTRODUCTION:Lipomasarecommonbenignmesenchymaltumorthatarisefromtheadiposetissue.

Differentialdiagnosisbetweenthebenignandmalignantform(liposarcoma)mustbemadetoprevent complicationandrecurrence.

PRESENTATIONOFCASE:Wereporteda63-year-oldmanwhopresentedwithrecurrentlarge-sizedmass ontherightforearmwiththeﬁrstpresentationapproximately28yearsbeforebeingtreatedinourcenter.

Maincomplaintswerediscomfort,socialembracement,anddifﬁcultyindressing.Therewerenocom- pressionsymptomsobserved.Weperformedmarginalexcisionandsentthesampleforhistopathological examination.TheworkhasbeenreportedinlinewiththeSurgicalCaseReport(SCARE)criteria.

DISCUSSION:Duringoneyearofpost-operativeperiod,norecurrencewasdetectedandacompleterelief ofsymptomswasasexpected.

CONCLUSION:Diagnosticchallengeinsuchalarge-sized(giant)lipomaisindifferentiatingitwiththe malignantform.Surgicalexcisionisthechoiceoftreatment.However,long-termfollowupisneeded duetotheriskofrecurrence.

1. Introduction

Lipomaisoneofthemostcommonbenignsofttissuetumors.

Itcommonlyoccurssubcutaneously,howeveritsappearanceon anyotherplanehasbeenreported.Large-sizedlipomaor‘giant lipoma’isconsideredifthesizeisgreaterthan5cmindiameter.

Wepresentedacaseofa63-year-oldmalewithgiantlipomaonthe ﬂexorcompartmentofhisrightforearmwhichwastreatedwith completeremovalofthelipomatousmasswithmarginalexcision.

Post-operativehistopathologicaldiagnosiswaswell-differentiated liposarcomalipoma-like.Norecurrencehadbeenobservedonthe 1-yearfollowup.OurworkhasbeenreportedinlinewiththeSur- gicalCaseReport(SCARE)Guidelines[1].

2. Case

A63-yearoldmalewasreferredtoorthopedicoutpatientclinic atourinstitutewithalargelumpontherightforearmregion.The lumpinitiallyappeared28yearsagoandhadre-appearedforthree timesafterthreetimesremoval.Thelatestremovalwas7years beforeadmittedtoourcenterandthelatestrecurrencewasinthe

∗Correspondingauthorat:DepartmentofOrthopaedicandTraumatology,Faculty ofMedicineUniversitasIndonesia,Dr.CiptoMangunkusumoHospital,Jl.Dipone- goroNo.71,Jakarta,10430,Indonesia.

E-mailaddress:[email protected](D.Aprilya).

next2years.Thepatientwasonlycomplaining aboutmild dis- comfort anddifficultyinwearingclothes.Thenon-tenderlump wasincreasinginsizeslowlyfromthesizeofquaileggswithwell- definedborderaroundthewristuntilitoccupiedthewholeflexor compartmentoftherightforearm.Therightforearmcircumference was20cmcomparedto7cmonthecontralateral(Fig.1).Therewas neitherneurologicaldeficitnorsignsofcompartmentsyndrome distaltothelump.

Thelaboratorymarkersshowednoabnormalityexceptforhigh bloodglucosesincethepatienthasdiabetesmellitussincethepast 11years.Healsohadahyperlipidemiawithincreaseofbothtotal andLDLcholesterollevel.

Patient underwent radiograph assessment with plain x ray whichsuggestlipomatousmass(Fig.2)andMagneticResonance Imaging(MRI)whichconcludedaprominentlipomatousmasson intermuscular region withpossible benign lesion or low-grade malignancy(Fig.3).OntheClinico-PathologicalConference(CPC), thismorelikelytobebenignlipomatouslesionandwasdecidedto undergosurgicalexploration,marginalexcisionandbiopsy.

Surgicalexploration was therefore performedunder general anesthesiaandwiththeapplicationofahemostatictourniquet.A lazy-Sdesignwasmadeapproximatelyonecmlateraltotheante- cubitalfossaandextended distallyuntildistal wristcrease.The incisionstartedontheulnar-volarsideonthedistalforearmwhere theinitialmassappearedandwhereitmostsuperﬁciallylocated undertheskin.Afterskinincisionandenteringthesubcutaneous planeonthewristregion,there wasalipoma-likelesionwhich https://doi.org/10.1016/j.ijscr.2020.05.063

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92 W.Widodoetal./InternationalJournalofSurgeryCaseReports72(2020)91–95

Fig.1. Lumponthevolarsideofrightforearm.

Fig.2.Amoreradiolucentsofttissuemasscomparedtosurroundingsonthevolarpartofrightforearmonthelevelofproximalshaftradius-ulnatometaphysealregionof distalradius-ulna.Corticalthickeningwasnotedonmid-shaftradius.Nobonydestructionorlesionswereobserved.

continuedsub-muscularlyunderneaththeFDSmuscle.Bluntdis- sectionthroughmusclefiberconfirmedadeep-seatedlipomalying overtheFlexorPollicisLongus(FPL)muscle.Runningthroughthe lipomawasashinycord-likestructure,whichwasidentifiedasthe mediannerve.Themasswascarefullydissectedoffthemediann.

untilreleasingthetransversecarpalligamenttoensurethatthe nervewasleftintact.Themasswasfoundtocompressthesur- roundings;however,itwasrelativelyeasytoremoveduetothe pseudo-capsuleandtheintermuscularlocation(Fig.4).

The15×8×5.5cmsmasswassentforhistopathologicalanal- ysis.Themasswaswell-vascularizedasobservedthattherewere

bigvesselsthatgavebloodsupplytothemassandthesevessels inﬁltratedthemassespeciallyatthesuperiorpole,whereitgave ayellow-redappearanceonthatregion.Themassconsistencywas generallyrubberywithaﬁrmerconsistencyatthesuperiorpole.

(Fig.5).

Histopathologicalanalysissuggestedalipomatoustumor,low grade,mostsuitablewithwell-differentiatedliposarcoma(WDL)- lipomalikesubtype(Fig.6).

During routinefollow-upin theoutpatientclinicatone and threemonths,thewoundhadhealedwellandtherewerenoneu- rologicaldeﬁcit,functionalimpairmentorrecurrenceofthelump

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Fig.3.Well-definedmarginmassliesinter-muscularlydeepintotheflexorcarpiradialis(FCR)muscleandcommondigitalflexorwiththesizeof20.2×5.1×3.6cm(a).

Surroundingstructureswerepressedwithoutanyinﬁltrationsobserved.OnT1Wsequencethisfattydominancelesionhashyperintensity(b)whichbecamehypointenseon STIRsequences(c).Thereweresimpleseptationswithinthemasswhichshowedcontrastenhancement.

Fig.4.Yellowishlipomatousmasswithpseudo-capsule.(a)Themasswassubcutaneouslylocatedonthewristregionwithproximitytothemediann.(arrow)and(b)became intermuscularasitextendeddeepintothemoreproximallocationontheforearmandoccupiedthespacebetweenFCRandFDCmuscle.(c)Thetumorwasdetachedfrom FPL(arrow)asitsdeepestadjacentstructure.(d)Finalclinicalpictureaftertumorremoval.

observed.Atone-year follow-upthescarhealed nicelywithout recurrenceofthemass(Fig.7).

3. Discussion

The etiology and pathogenesis of lipomas remain unknown, althoughgeneticendocrineandtraumaticfactorshavebeensug- gested. Our patienthad diabetes mellitus in thepast 11 years whichwastreatedbyoralanti-diabeticdrugs.Healsohadalong- standinghyperlipidemiawiththeincreaseoftotalcholesteroland LDLcholesterollevel.

Typical lipoma occurs more often in young adults, women (thigh),andinoverweightpopulations.Theyvaryinsize[2];when thesizeisgreaterthan5cmindiameter,theterm‘giantlipoma’is

considered,asitmayraisetheawarenesstoothermalignantformof lipomatousmasssuchasliposarcoma[3].Ourpatientisa63-year- oldnormal-weightmanwhichhadﬁrstpresentationofthelipoma approximately28yearsago;thisisanuncommoncharacteristic forthetypicallipoma.Furthermore,itspresentationinvolarpart oftheforearmisalsounusualfromthoseofgiantlipomaswhich occurmostfrequentlyincervicalregion,thorax,andlowerlimbs (inguinalregionandthigh)[4].

Althoughthedeﬁnitivediagnosisofgiantlipomacanbemade onlybyhistopathologicalexamination,thedifferentialdiagnosis between giant lipoma and liposarcoma is of great importance.

Oncesuspected,otherinvestigationsmustbeconductedtoprovide moreinformationaboutthetumorsuchasultrasonography(USG), computedtomography(CT)scan andmagneticresonanceimag-

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94 W.Widodoetal./InternationalJournalofSurgeryCaseReports72(2020)91–95

Fig.5. Lobulated,wellcircumscribed,well-vascularizedandrubberyyellowishmasswithaﬁrmerareaonitssuperiorpole.Therewerenofatnecrosisorhemorrhage.(a) beforedivided,(b)innerside,and(c)outerside.

Fig.6.HistopathologicalpictureinHEstainingwithvariousmagniﬁcation.Therewasadiposecellproliferationwithvaryingsizeandscatteredatypicalcells,ofwhich,some hasnucleoli.(a)40×,(b)100×,and(c)400×magniﬁcation.

ing(MRI)aswellastechnetium-99diethylenetriaminepentaacetic acidscanning[2,3,5].

Inourcase,theplainradiographshowedaradiolucentsofttis- suemassalongtheshaftofradiusulnaonthevolarpartofthe rightforearm. The MRI showedhomogeneoushyperintense fat signalonT1-weightedMRimageswhereas T2-weightedimages showedhypointensefat-suppression signal.Themass appeared inter-muscularlybetweenFCRandFDCwithwell-deﬁnedmargin andnoinﬁltrationtosurroundingstructures,raisingthesuscepti- bilityofalipomatoustumorwithbenigncharacteristics.

Surgical is the mainstay treatment for giant lipoma due to thesize,recurrenceriskandpotentialmalignanttransformation.

Completesurgicalresection,withpreferablywide-margin,isrec- ommendedtopreventdedifferentiationandrecurrence.Adjuvant radiation therapy maybeneﬁt in large high-grade liposarcoma andinanyotherliposarcomawhenthewideresectioncannotbe obtained.Thevalueofchemotherapyisstilldebatableasadjuvant tosurgicaltherapy[6].

Inourcaseduetothecomplicatedstructureandrelativelynar- rowspaceattheforearm,wecouldnotperformwideresectionwith muchnegativemargins.Intraoperatively,wefoundthatthemass wasdeep-seated,well-vascularized,well-circumscribed,lobulated andhadarubberyconsistency.Thosecharacteristicsweresuitable forthelipomatousmassbutnotspeciﬁcallypresentliposarcoma.

Thecutsurfacewasyellowwithmorehyperemicareasonthesupe- riorpoleofthemass.Apseudo-capsulewhichwasformedbythe continuouspressureonsurroundingtissuemadethedissectionand enucleationofthemassuncomplicated.Sincethemasswasinter- muscular,wewereabletoremove thetumorwithoutaffecting

theadjacentmusculartissuewhilepreservingtheneurovascular bundle,inthiscase,themediannerveparticularly[3,4,7].

Post-operativehistopathologicalexamrevealedthisgiantlipo- matous mass as the well differentiated liposarcomas (WDL) lipoma-likesubtype.Accountfor40–45%ofliposarcomas,WDL usually diagnosed at the 5th decades of life with slight male predominance.WDLarelocallyaggressivemesenchymaltumors composedofmature adipocytesandstromal cells withatleast focalcytologicatypia.WDLareconsideredaslowgradelipogenic tumorswithmorphologicsubtypesdependentontheadipocytic componentandbackgroundcellularizationcharacteristic.Several histologicsubtypeshavebeendescribed:lipoma-like,sclerosing, inﬂammatory,mixed,lipo-leiomyosarcoma,WDLwithlowgrade osteosarcoma-likeandspindlecellsubtype.Lipoma-likesubtype, beingthemostcommonformofWDL,isgrosslyindistinguishable fromlipoma.Itfrequentlycontainslipoblastandscatteredatypical cellsmaybediffuseorextremelyrare[7–9].

Inthisreport,surgicalexcisionresultedincompletereliefof symptoms and signiﬁcant esthetic also became evident in the affectedforearm.Therewasnorecurrenceinthe1-year follow up.However,westillcloselyobservethispatientforanyrecur- rencesincethepossibleriskofrecurrenceintheWDLaswellas inter-muscularlylocatedlipomatousmass.

4. Conclusion

The slow growing nature and radiological characteristic of lipomabroughtconfusionwiththebenignlesion. However,the giant size and otherunusual characteristics (gender, age,mass

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Fig.7. Clinicalappearanceatoneyearfollowup.

location and recurrences) raised the awareness of malignant lipomatous mass. Appropriate diagnosis and complete surgical resection of this tumor provided symptoms relief and esthetic improvementontheaffectedlimb.Long-termevaluationmustbe conductedinordertodetectrecurrence.Weplanforthewideexci- sionfollowedbyradiotherapyforpossiblerecurrencemassinthe future.

DeclarationofCompetingInterest

Theauthorsdeclarenoconﬂictsofinterest.

Funding

Theauthorsreportnoexternalsourceof fundingduringthe writingofthisarticle.

Ethicalapproval

Ethicalapprovalwasnotrequiredinthetreatmentofthepatient inthisreport.

Consent

Writtenconsenthasbeenreceivedfromthesubject.

Authorcontribution

DinaAprilyacontributestothestudyconceptordesign,data collectionandwritingthepaper.

WildanLatiefcontributesinthestudyconceptordesign,data collection,analysis andinterpretation,oversight andleadership responsibility for the research activityplanning and execution, includingmentorshipexternaltothecoreteam.

Wahyu Widodo contributes in the study concept or design, datacollection,analysisandinterpretation,oversightandleader- shipresponsibilityfortheresearchactivityplanningandexecution, includingmentorshipexternaltothecoreteam.

Registrationofresearchstudies Notrequired.

Guarantor

WahyuWidodoisthesoleguarantorofthissubmittedarticle.

Provenanceandpeerreview

Editoriallyreviewed,notexternallypeer-reviewed.

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