Giant retroperitoneal liposarcoma: Case report and review of the literature

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InternationalJournalofSurgeryCaseReports9(2015)23–26

ContentslistsavailableatScienceDirect

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

Giant

retroperitoneal

liposarcoma:

Case

report

and

review

of

the

literature

Antonio

Caizzone,

Edoardo

Saladino,

Francesco

Fleres

,

Cosimo

Paviglianiti,

Francesco

Iaropoli,

Carmelo

Mazzeo,

Eugenio

Cucinotta,

Antonio

Macrì

InstitutionandDepartmentofHumanPathology,UniversityofMessina,ViaConsolareValeria,98125Messina,Italy

a

r

t

i

c

l

e

i

n

f

o

Articlehistory: Received14May2014

Receivedinrevisedform9February2015 Accepted12February2015

Availableonline17February2015 Keywords:

Retroperitonealliposarcoma Softtissuesarcomas Myxoidareas Radicaltreatment

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AIM:Retroperitonealsoft-tissuesarcomasarerelativelyuncommondiseases,themostfrequent histo-type,rangingfrom20%to45%ofallcases,isrepresentedbyliposarcoma,whichisahard-totreatcondition foritslocalaggressivenessandclinicalaspecificity.

PRESENTATIONOFCASE:Wereportacaseofa64-years-oldwomanwhounderwentsurgicalresectionfor agiantpleomorphicretroperitonealliposarcoma.

DISCUSSION:Currentlychemotherapyforretroperitonealsoft-tissuesarcomasisnoeffective,and radio-therapyhaslimitedefficacyduetothetoxicityaffectingadjacentintra-abdominalstructures,showed validityonlyincaseofhigh-grademalignancybyreducinglocalrecurrence,butwithnoadvantagein overallsurvival.Nowadaysonly,thecompletesurgicalresectionremainsthemostimportantpredictor oflocalrecurrenceandoverallsurvival.

CONCLUSION:Theremovalofaretroperitonealsarcomaofremarkablesizeisachallengeforthesurgeon owingtotheanatomicalsite,totheabsenceofananatomicallyevidentvascular-lymphaticpeduncle andtotheadhesionscontractedwiththecontiguousorgansandwiththegreatvessels.Therefore,we believethat,particularlyforlarge-sizediseasesassociatedtohigh-grademalignancy,acompletesurgical resectionwithremovalofthecontiguousintraandretroperitonealorganswheninfiltratedrepresents theonlytherapeuticoptiontoobtainanegativemarginandthereforeanoncologicalradicality.

©2015PublishedbyElsevierLtd.onbehalfofSurgicalAssociatesLtd.Thisisanopenaccessarticle undertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/).

1. Introduction

Amongtheretroperitonealsarcomas,thatrepresentthe10–15% ofallsofttissuesarcoma[1],themostcommonhistotypeis repre-sentedbyliposarcoma,whichrangesfrom20%to45%ofallcases

[2]. It commonly occursin patientswith 40–60years-old with a 1:1ratio betweenmaleand female[3].Theliposarcomamay haveweightanddimensionvariable;thoseover20kgarecalled “giant liposarcomas” and are extremely rare [3]; someauthors havereportedmassesbetween18and46.6kg[4–6].Wereporta rarecaseofagiantretroperitonealliposarcomaevenmore uncom-monforitsoriginfromperirenalfat:infactapproximately13%are locatedintheretroperitonealarea,andlessthan1/3ofthesearise fromperinephricfat[7].

∗ Corespondingauthor.Tel.:+390902212678/3474062416; fax:+390902212633.

E-mailaddress:franz.fl[email protected](F.Fleres).

2. Casereport

Wereportacaseofa64year-oldwomanthatwasreferredtoour observationforaprogressivevolumetricincreaseoftheabdomen. Thecomputedtomography(CT)oftheabdomendemonstratedthe presenceofavoluminousmass,extendingfromthesub-hepatic spaceuptothepelviccavitywithdislocationoftherightkidney totheleft(Figs.1and2).Themassappearedasmixedstructure, characterizedbythecoexistenceofareaswithdifferentdensity, adiposewiththickseptaandsolidwithsuperfluiddensity,with cranio–caudalextensionofover30cm.Thereforethepatientwas submittedtoanexplorativelaparotomywhichrevealedthe pres-enceofabulkylesionwithamultinodularappearance,originating fromtherightretroperitonealregion.Thelesionhadproduceda remarkabledislocationofintra-andretro-peritonealorganstothe leftsidedislocating pancreas,kidneyand wholeintestinalmass toaspacebetweentheleftflankandhomolateraliliacfossa.For theapparentinfiltrationoftherightkidney(Fig.3)wasperformed abiopsythatconfirmeditsneoplasticinfiltration.Weproceeded torelease theneoplasm fromtheadhesions byretroperitoneal contiguousorgansincludingthevenacavaandcommoniliac ves-sels, withitssubsequentremoval enbloc withtherightkidney

http://dx.doi.org/10.1016/j.ijscr.2015.02.019

2210-2612/©2015PublishedbyElsevierLtd.onbehalfofSurgicalAssociatesLtd.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense

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Fig.1.CTscanshowingthepresenceofthebulkylesionstretchingfromthe sub-hepaticregiontothepelviccavity,displacingtherightkidneytotheleft.

Fig.2.CTscanshowingthemacroscopicintralesionalcharacteristics,consistingof thepresenceofareaswithdifferentfatdensityandthickseptaassociatedwithan inhomogeneoussolidcomponentfeaturingsovrafluid-densitycoarsecomponents.

Fig.3.Evidenceofthecloserelationshipbetweenthelesionandtherightkidney andtheoriginfromtherightretroperitonealregionattheleveloftheipsilateral parietocolicaspace.

(Fig.4).Thepostoperativecoursewasuneventfulandthepatient wasdischargedonthe3thpostoperativeday.Thedefinitive histo-logicaldiagnosiswaspleomorphicliposarcomawithmyxoidareas, (42×37×18cm)(Fig.5),originatingfromtherightperirenalfat andinfiltratingtheperiureteraltissue,withfreeresectionmargin. At24monthsoffollow-upthepatientisdiseasefree.

Fig.4.Rightretroperitonealcavityasitappearedoncethelesionhadbeenremoved andtherightnephrectomyhadbeenperformed,withevidenceofthestructuresthat hadcloserelationsofcontiguitywithit.Theinferiorvenacavaiseasilyrecognizable aswellastheiliopsoasmuscle,gonadalbloodvesselsandrightiliacvessels.

Fig.5. Pictureoftheremovedsurgicalspecimenincludingtherightkidney.

3. Discussion

Theretroperitonealliposarcomasaregenerallyneoplasmswith a low or intermediate grade of malignancy. The occurrence of hematogenousmetastasisisararefindingatthetimeof diagno-sis;thelung representsthemain siteofdistant metastases[8]. Fromahistologicalpointofview,inaccordingtothe morphologi-calcharacteristicsandonthestrengthofcytogeneticaberrations, nowwidelyaccepted,wecandividetheliposarcomain4types[8]: (1)undifferentiated,(2)pleomorphic,(3)welldifferentiated,(4) myxoid/roundcell.Theundifferentiatedandpleomorphictypeare neoplasmwithhighgradeofmalignancyaccompaniedby remark-ablebiologicalaggressivenessandwithmetastaticpotentialwhile well-differentiatedandmyxoid/roundcellformsaretumourswith alowgradeofmalignancy,associatedwithamorefavourable prog-nosis [9]. The well-differentiated and undifferentiatedtumours representthemostcommonbiologicalgroup.Theformeris char-acterizedbylocalaggressivenesswithlowmetastaticpotential,its

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A.Caizzoneetal./InternationalJournalofSurgeryCaseReports9(2015)23–26 25 clinicalmanifestationoccursthroughthecompressionofadjacent

organsorstructures,andtotheCTimagingusuallyappearsasa homogeneouslesionwiththesamedensityofadiposetissue,well encapsulated,withthepresenceofthicksepta.Instead,the lat-terusuallyoriginatesfromahistologicalaberrationoccurringin thecontextofawell-differentiatedliposarcoma.Thefurtherloss ofdifferentiationdeterminesacellulartransformation,which is evenmorecharacteristicoftherelapseofdisease,itoccursin20% atthefirstrecurrenceandin44%atthesecondone[2].TheCT imagingoftheundifferentiatedsarcomademonstratesa hetero-geneous,nolipogenicmass[8,10].Incaseofdiagnosticdoubtand inpresenceofrecurrence,MagneticResonanceImaging(MRI)may beusefulbecausecanidentifyinginareliablemannerthesatellite localizationsofthemainlesion.Theresectionofaretroperitoneal sarcomaofremarkablesizeisachallengeforthesurgeonowing totheanatomicalsite,totheabsenceofananatomicallyevident vascular-lymphaticpedunclethatmakesithardtoobtainsafe mar-ginandtotheadherenceswiththecontiguousorgansandwiththe greatvessels.Thereforetheretroperitonealliposarcomashowsa highrateoflocalrecurrenceaftersurgicalexcision.Actually,the completesurgical(R0)resectionrepresentstheonlypossibilityof radicaltreatment,infactasreportedinastudy[11]carriedout on177patientswithretroperitonealliposarcomaoperatedwith curativeintent,thepercentageofpatientsdiseasefreeat3and5 yearswas73%and60%respectively.Theprognosticfactors sta-tisticallyassociatedwithsurvivalwerefoundtobethehistotype andthetypeofresectionperformed(completevs.partial).Actually theoverallsurvivalat5-yearsreportedinliteratureforthevarious histologicalsubtypeswelldifferentiated,myxoid/roundcell, undif-ferentiatedandpleomorphic,rangingfrom90%,60to90%,75%and 30to50%,respectively[11].

Theresectionofneighbororgansisusuallyrequiredto facili-tatedissection,butcanbeessentialtogetaradicalmacroscopic removal,thatsignificantlyinfluencetheprognosis.Neuhausetal.

[12] reportedastudy carriedout on190patients, of whom72 weresubmitted tocurative surgery for retroperitoneal liposar-coma(RPLS),andother47hadundergonepalliativeresectionfor recurrentRPLS.Overhalfofthepatientsunderwentresectionwith curativeintentionhadexcisionofacontiguousorgantoachieve macroscopicclearanceatprimarysurgery.However,organswere directlyinfiltratedbytumourinonly4%ofpatients.However,asin ourpatientaboutthe15%ofretroperitonealliposarcomasoriginate fromtheperirenalfat[7,13].Asinourcasereport,thekidneysare oftendislocatedorrotatedbythemassandcanbethesiteof neo-plasticinfiltrationwithpossibleonsetofpyelo-ureteralnephrosis. Consequentlytheyaretheorgansmostinvolvedintheresection, immediatelyfollowedbythecolon[14].Followingsurgical resec-tion,the 50 - 100% of liposarcomasrecur fromresidual tissue, whichistheprimarycauseofdeath[1].Thereforeanaggressive surgicalbehaviourisjustified,withtheresectionofthestructures andviscera adjacenttothepathologicalprocess intheattempt toobtainfreediseasemarginallowingtoobtaina5-year recur-rencerateof22%thatresultsneverthelessinanincreasemorbidity respecttothepast[12,15,16].Infactforthis reasonbeing diffi-culttodiscriminateintraoperativelythepathologicaltissue,from thosenormal,thetumoursshouldberesectedbyincludingamore abundantquantityofretroperitonealfat.However,theresectionof organsorstructuresinvadedbythetumour,althoughinfrequent, shouldoccuronlyincaseinwhichisnotpossibletoidentifyasafe planeofdissectionbetweenthetumourandtheorgansadjacent toit,withanenblocresection.Lewisattributesaperi-operative mortalityof 4%identifying haemorrhage, sepsis, acute myocar-dialinfraction,andmultiorganicfailureastheprincipalcausesof death[2].Actuallythechemotherapytreatmentusedinthe adju-vantorneoadjuvantsettinghavenobenefitintheclinicalcourse ofthediseaseandthereforeisnoutilizedinaroutinemanner[17].

Doxorubicinonly yieldsa response rateof 18–29%.The antibi-oticsalinomycin,apotassiumionophore,appearstoincreasethe chemosensitivitytothedoxorubicin;soitmaybeusedtodecrease thedoxorubicindosageand itstoxicsideeffects [18]. Neoadju-vanttherapymaybetookintoaccountincasesofdedifferentiated liposarcoma(DDLS),whichhasanincreasedriskofrecurrenceand metastasis.It’snecessaryanaccuratesubtype-specificdiagnosisto evaluatetheneoadjuvanttherapy.Inaretrospectivestudyof120 patientswhounderwent137preoperativepercutaneousbiopsies followedbysurgicalresections,Ikoma[19]hasdemonstratedthat percutaneousbiopsyhaslowaccuracyinthediagnosisof retroperi-tonealDDLS.Thiscanpotentiallymisleadthedecisiontoadoptthe neoadjuvanttreatment.

Someretrospectivestudieshaveunderlinedthebenefitsofthe adjuvantradiotherapy,usedintumourswithadiametergreater than5cmandpositivesurgicalmargin,withbettercontroloflocal recurrencebutwithoutincreasesinlong-termsurvival[20]. 4. Conclusion

Our case shows somepeculiar characteristics: firstlyits ori-gin fromperirenal fat, infact approximately 13% are locatedin the retroperitoneal area, and less than 1/3of thesearise from perinephricfat;and secondlythehuge size(42_×37_×18cm)of liposarcoma,soitcanbedefinedgiant.

Actuallythesurgicalapproachrepresentstheonlytherapeutic optionthatcanprovideaconcreteperspectiveofcareconsidering lackofsupportofeffectivecomplementarytherapies.Anaggressive surgicalattitudethatincludesextendedresectionsoftheextra-and intaperitonealstructuresisjustified,especiallyintumoursoflarge size,inordertoobtainamicroscopicradicalityofresectionmargins. Conflictofinterest

Allauthorsdeclarethattheyhavenotanyconflictofinterest. Funding

Theauthorsdeclaretherearenotanysponsorsinvolvement. Ethicalapproval

Theauthorsdeclarethatallproceduresfollowedwerein accor-dancewiththeethicalstandardsoftheresponsiblecommitteeon humanexperimentation(institutionalandnational)andwiththe HelsinkiDeclarationof1975,asrevisedin2008.Informedconsent wasobtainedfromthepatientforbeingincludedinthestudy. Consent

Authorsdeclarethattheyhaveobtainedwritteninformed con-sent from the patient for publication of this case report and accompanyingimages.Acopyofthewrittenconsentisavailable forreviewbytheEditor-in-Chiefofthisjournalonrequest. Authorcontribution

Antonio Caizzone concept or design, data collection, data analysisorinterpretation,writingthepaper.EdoardoSaladino con-tributor.

FrancescoFlerescontributor,correspondingauthor,translator. CosimoPaviglianiticontributor.

FrancescoIaropolicontributor. CarmeloMazzeocontributor. EugenioCucinottacontributor.

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AntonioMacrìstudyconceptor design, datacollection,data analysisorinterpretationandreviewer.

Guarantor AntonioCaizzone. EdoardoSaladino. FrancescoFleres. AntonioMacrì. References

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