Primary renal osteosarcoma: A case report

AfricanJournalofUrology(2014)20,189–192 HOSTED BY

Pan African Urological Surgeons’ Association

African Journal of Urology

www.ees.elsevier.com/afju www.sciencedirect.com

Primary renal osteosarcoma: A case report

C. Ahomadégbé

, N. Bennani-Guebessi , M. Karkouri

PathologyDepartment,CHUIbnRochdCasablanaca,Morocco

Received7June2014;receivedinrevisedform25July2014;accepted7August2014

KEYWORDS Extra-osseous osteosarcoma;

Kidney;

Primarytumor;

Poorprognosis

Abstract

Primitiverenalosteosarcomaisararesarcomaofthekidneywithonly27casesreportedintheliterature.Its histogenesisispoorlyunderstood.Itoccursatanolderagebetweenthefifthandseventhdecadeoflifewith amalepredominance.Theclinicalfeaturesaresimilartootherrenaldiseases.Imagingshowscalcifications withinalumbarorflankmass.Histologydescribesasarcomatousproliferationproducingosteoid,most oftenatanadvancedstage(pT4),whichimpliesapoorprognosis.Wereportontheclinicalandpathologic featuresofacaseofprimaryrenalosteosarcomaina56-year-oldmanwithstageIVdisease.Thisisthe 28thcaseofprimitiverenalosteosarcomareportedintheliterature,confirmingthehighlymalignantnature ofthistumorandtheneedforearlydiagnosis.

©2014PanAfricanUrologicalSurgeons’Association.ProductionandhostingbyElsevierB.V.Allrights reserved.

Introduction

Extra-osseousosteosarcomaisararemalignanttumorrepresenting 1–2%of softtissuesarcomasand lessthan4%ofosteosarcoma [1,2].It growsoutside ofthe boneskeletonandis composedof malignantosteoblasticcellsproducingboneorcartilagematerial.

Its traditional locationsareatthe soft parts ofthe limbsand in the retroperitoneum [3,4]. Other rare locations, however, have been described, among these the testicles [5], hand [6], brain, mediastinum,diaphragm,lungandheart[1].Thekidneyisalsoa raresitetobeaffectedbyextra-osseoussarcomawith,todate,only 27casesreportedintheliterature.Wereportanadditionalcaseof primaryrenalosteosarcomaina56-year-oldman.

∗Correspondingauthor.

E-mailaddress:christelle.ahomadegbe@gmail.com(C.Ahomadégbé).

PeerreviewunderresponsibilityofPanAfricanUrologicalSurgeons’

Association.

Casereport

A56-year-oldmanwasadmittedwithapainlessleftflankmassand aseven-monthhistoryofintermittenttotalhematuriawithoutweight loss.Physicalexaminationrevealedalargenon-inflammatoryleft lumbarmass extending to the flank and the hypochondriac and periumbilicalregions.Urographydisplayedacalcifiedleftkidney tumorsized28cmwithdilatationoftherenalpelvisandcalyx.The corticalindexwaslessthanonemillimeter.Laparoscopic radical nephrectomywasperformed.Grossexaminationshowedanecrotic, hemorrhagicandpolychromesolid andcysticneoplasmmeasur- ing23×14×13cm(Fig.1).Ithadcompletelydestroyedtherenal parenchymaandinfiltratedtherenalcapsuleandperirenalfatwith- outinvolvementoftheadrenalgland.Histologicalexaminationof 23sectionsintotal(onesectionpercentimeteroftumor)showed alargelynecroticandinfiltratingmalignantproliferationconsist- ingofsheetsofpleomorphiccells,globular,spindle-shapedorgiant multi-nucleatedosteoclast-typecellswithvariableamountsofcal- cifiedosteoidproductionandfociofcartilaginous differentiation 1110-5704©2014PanAfricanUrologicalSurgeons’Association.ProductionandhostingbyElsevierB.V.Allrightsreserved.

http://dx.doi.org/10.1016/j.afju.2014.08.004

190 C.Ahomadégbéetal.

Figure1 Grossexamination(A)Renalnodularformationmeasuring23×14×13cm.(B)Twocutsections:necrotic,hemorrhagicsolidand cysticneoplasm.

(Fig.2).Therewasabreachoftherenalcapsuleandinfiltration oftheperirenalfat.Thehilumwasalsoinfiltratedbythe tumor.

Immunohistochemicalexaminationrevealednopositivecytokeratin reaction,butheterogeneousexpressionofmuscle-specificactinby theneoplasticcells(Fig.3).TheneoplasmwasdiagnosedaspT4 high-gradeprimaryosteoblasticosteosarcomaofthe kidney.The outcomewasunfavorable;thepatientdiedonemonthaftersurgical excisionofthetumor.

Figure2 Hematoxylin-eosinstain(A)Magnification×20,osteoid productionbyneoplasiccells(arrows:osteoidproduction).(B)Mag- nification×40,malignantproliferationmadeofsheetsofpleomorphic cells(arrow:pleomorphiccells).

Discussion

Sarcomasaccountforapproximately1%ofprimaryrenalmalig- nanciesinadults[7].Differenttypesofprimaryrenalsarcomahave beendescribed,suchasEwing’ssarcoma[8],primaryneuroectoder- maltumors[9],synovialsarcoma[10]andleiomyosarcoma[11].

Thelatteris the mostfrequentprimitive renalsarcomareported withinthisgroup[7,11].Renalosteosarcomaisarareentitywith, todate,only27reportedcasesintheliterature[12].Themale-to- femaleratioinprimaryrenalosteosarcomais2:1,withthetumor occurringmorefrequentlyduringthefifthtoseventhdecadeoflife [12].Theclinicalsymptomsaresimilartorenalpathologiesingen- eralandshownospecificity.Theymayconsistofapalpableflank mass,lumbarpain,weightloss,and,rarely,grosshematuria.Our patienthadaninsidioussymptomatologyjust presentingastotal grosshematuriawithoutothersigns.Asinthepresentcase,calcifi- cationsarefoundwithinthetumorinthemajorityofreportedcases [13].ThecurrentWorldHealthOrganization(WHO)classification ofurogenitaltumorsdefinesosteosarcomaasaproliferativepro- cessinwhichtheneoplasticcellsproduceosteoidinstroma[14].

Itsphysiopathologyremainsunclear.Thesecellshavetheabilityto differentiateintofibroblastic,chondroblasticandosteoblasticcells according tothe classicVirchowtheory regarding the metaplas- tictransformationofconnectivetissueintoprimitivemesenchyme withtheabilitytodifferentiateintoosteoblasts[15].Histologically, osteosarcomacorrespondstoasarcomatousproliferationproduc- ingosteoid.Pleomorphicosteosarcomaisthepredominantsubtype (40%);theosteoblasticandchondroblasticsubtypesaredescribed less frequently[12]. Inour case,it wasanosteoblasticsubtype.

Immunohistochemistryisoflittlevalueinthediagnosisbecauseit showsnospecificity,butitcanremovetheambiguityofacarcinoma [16].Infact,someauthorssuggestarelationshipbetweenosteosar- coma and carcinosarcoma where the mesenchymal component overgrowstheepithelialcomponentandvirtuallymakesitdisappear [17].Incaseswithprovenpositivityforcytokeratin,evenfocally, thetumorismostlikelytobeasarcomatoidrenalcellcarcinoma (carcinosarcoma)withapredominantosteosarcomatouscomponent orwiththecarcinomacomponentovergrownbythesarcoma[17].

As sarcomatoidcarcinomas are farmore commonthan primary osteosarcoma,caremustbetakentoensurethecorrectdiagnosis.In ourcase,alargenumberofsamplesfromthetumorwereexamined.

Asthemalignantcellsdidnotexpresscytokeratin,acarcinomatous componentcouldbeexcluded.However,focalmuscle-specificactin expressionwasseenwhichcomplieswiththefindingsofotherstud- ies[18].Likeinourcase,allthereportsintheliterature(withone

Primaryrenalosteosarcoma 191 exception)describedahigh-gradeprimaryrenalosteosarcoma,with

stagepT4atdiagnosisinabout60%[12].Likeotherrenalsarco- mas,primaryrenalosteosarcomaisahighlymalignanttumorwitha poorprognosis,usuallywithamediansurvivaltimeof8–22months atdiagnosis[12,14].Localrecurrenceandmetastasesarefrequent in tumorsaffecting the peritoneum,bonemarrow,lung, boneor liver [11,19–21]. Metastasis ossification has also been reported [11,19–21].Thedifferentialdiagnosisofprimaryosteosarcomaof thekidneyincludessarcomatoidrenalcellcarcinoma,adultWilm’s tumor,metastaticsarcomaandsarcomatoidurothelialcarcinomaof therenalpelvis,whichoftenpresentsatanadvancedstageandmay haveosteosarcomaasaheterologouscomponent[11,12,22].

Inconclusion,primaryrenalosteosarcomaisararetumorwitha poorprognosis.Its clinicalsymptoms,sometimes insidious,can delaydiagnosis.Theclinicalfeatures,thechronologyofthelesions, a good tumor sampling and immunohistochemistry (cytokeratin AE1/AE3) canhelp in the differentiation betweena carcinoma and a sarcomaof the kidney [7,23–25]. Our case of anelderly

Figure3 Immunohistochemistry(A)Muscle-SpecificActinstain, magnification×40,Heterogenousexpressionbytumoralcells(arrow).

(B)CytokeratinAE1/AE3stain,magnification×10,noexpressionby neoplasiccells.

patientpresentingwithtotalhematuria,whicheventuallyledtothe diagnosisofahigh-gradeprimaryrenalosteoblasticosteosarcoma, is the 28th case of primary renal osteosarcoma reported in the literatureand underlines the need of an early diagnosisof this highlymalignanttumor.

Conflictofinterest

Theauthorsreportednoconflictofinterestinthiswork.

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