Renal liposarcoma: case report and review of systemic treatment

Patricia Rioja1, Guillermo Valencia1, César Centurión-Rodriguez1, Zaida Morante1, Mercedes Bravo2, Lourdes Huanca2 and Carlos Morante3

1Medical Department, Instituto Nacional de Enfermedades Neoplásicas, Lima 15038, Peru 2Pathology Department, Instituto Nacional de Enfermedades Neoplásicas, Lima 15038, Peru 3Surgical Oncology Department, Instituto Nacional de Enfermedades Neoplásicas, Lima 15038, Peru

Abstract

Liposarcomas are malignant mesenchymal tumours usually located in the retroperito- neum, rarely occurring as a single lesion in the . We present a case of a 59-year-old male patient with a left renal mass detected by computed tomography scan. He under- went radical and the histopathological study reported a primary undifferen- tiated liposarcoma of the kidney without nodal involvement. After 15 months of surgery, he remained asymptomatic and without evidence of disease recurrence. The objective of this report is to present a case and literature review with current evidence of treatment options and prognostic factors for survival.

Keywords: liposarcoma, kidney , nephrectomy Case Study

Introduction

Liposarcomas are malignant neoplasms of mesenchymal origin that belong to the group of soft tissue , constitute less than 1% of all malignant tumours and have a high infiltrative and metastatic potential [1]. They occur most frequently in the extremities (52%) and in the retroperitoneum (19%). The treatment of choice is complete surgical Correspondence to: César Centurión-Rodriguez resection. The 5-year survival rate for a histologically well-differentiated liposarcoma is Email: [email protected] 83%, while for an undifferentiated one, it is about 20% [2]. In contrast, the prognosis of ecancer 2021, 15:1173 renal liposarcoma is poor, with a 5-year survival of 29%–39% after surgery [3]. https://doi.org/10.3332/ecancer.2021.1173 Renal liposarcoma is an extremely rare (1%–2% of primary renal sarcomas). It Published: 14/01/2021 belongs to the group of sarcomas of the genitourinary tract, of which leiomyosarcomas Received: 25/06/2020 are the most common, followed by liposarcomas. Liposarcoma of the kidney tends to be Publication costs for this article were supported by diagnosed incidentally in patients who present with renal mass, thoraco-lumbar pain and ecancer (UK Charity number 1176307). dyspnoea. Radical nephrectomy is the treatment of choice [4]. Copyright: © the authors; licensee ecancermedicalscience. This is an Open Access article distributed under the terms of the Aim Creative Commons Attribution License (http:// creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and The aim of this article is to report a rare case of a patient who was diagnosed as hav- reproduction in any medium, provided the original ing undifferentiated renal liposarcoma based upon pathology examination of his radical work is properly cited.

ecancer 2021, 15:1173; www.ecancer.org; DOI: https://doi.org/10.3332/ecancer.2021.1173 1 (perioperative, postoperative or advanced disease) andprognostic factors that have beendocumented to berelated to survival of patients. nephrectomy specimen,andto provide anupdated review of theliterature description of renal liposarcoma, itstreatment according to scenarios ecancer 2021, 15:1173; www.ecancer.org; DOI:https://doi.org/10.3332/ecancer.2021.1173 alteration of theadjacent fat planesinitscaudalpole. Figure 1.Computed tomography scan.(a): Abdominal CT scanshowing aneoplasticlesionintheleft kidney of heterogeneous density and (b): with vascular invasion by thetumour. There was noevidence of metastaticspread toGerota’s the fascia, renal pelvis,adrenal glandsas well asthere was no evidence of lympho- liposarcoma of highhistological grade, with 35mitoses per ten high-power field(35/10high-power fieldmitoses) and30%of necrosis. renal fat, nuclei and high mitoticwith pleomorphic index (Figure 3 ). These findings were reported to be consistent with an undifferentiated detected acell proliferationof tumour cells that were arranged inastoriform pattern which infiltrated therenal sinus,capsuleandtheperi- areas of myxoidnecrosis andhaemorrhagic ofrespectivelyand 10%, 30% (Figure 2).Microscopy pathology examination of thespecimen which was noted to have asolidcomponent, and which was softin consistency, and which was light brown incolour and which contained Macroscopy pathology examination of the specimen was reported to have identified a left renal tumour that measured 23 × 12 × 10 cm noted to have aleft kidney dependent tumour that measured 20×19cm with associated hilar andpara-aortic lymphnodes. In October 2019,heunderwenta surgery (leftradical nephrectomy pluspara-aortic lymphadenectomy), andduringtheprocedure he was considered.diagnosis was defined borders and contours and which was not associated with any nodal or vascular involvement (Figure 1 ). Kidney presumptive He hadcomputed tomography (CT)ofscan abdomen showedwhich aleft renal massof heterogeneous density of 19×129cm, which had moderate anaemia with ahaemoglobin of 9.0g/L andhyperkalaemia potassiumwith aserum of 5.8mmol/L. definedmargins, locatedfrom 12cm his left ribcage. The resultsof hisroutine haematology andbiochemistry laboratory bloodtests revealed of 5kg,early about feeling ofand night fullness sweating of 8months of evolution. His physical examination revealed amobilemass without A 59-year-old malepatient, from Peru, no relevantwith history, medical presented inSeptember 2019 ahistorywith of backpain, weight loss Case report 2

Case Study ecancer 2021, 15:1173; www.ecancer.org; DOI:https://doi.org/10.3332/ecancer.2021.1173 results of hisclinicalexamination, laboratory bloodtests andCT scanimages. He will continue to have regular follow-upassessments. After 15monthsof follow-up,the patient has remained well noevidencewith of localrecurrence or distant metastases baseduponthe undergoing regular follow-up assessments with clinicalexamination, laboratory tests andradiology imagingassessments. (radical nephrectomynode excision) and lymph asaprimary treatment with negative surgical resection margins (R0). The patient hasbeen of theleftkidney wasand thistumour made wasas clinicalstage classified IIIB(T4N0M0G3)for hehadundergone radical surgical excision expression cellsthe neoplastic within (Figure 3), did confirmwhich theproposed diagnosis. A definitive finaldiagnosisof renal liposarcoma Immunohistochemistry stainingstudy of thetumour showedMDM2 with that thetumour hadexhibited positive nuclear immunostaining immunoexpression of MDM2intumour cells confirmed thediagnosis of dedifferentiated liposarcoma (IHC100×). areas of abrupt andfocal transition from well-differentiated adipocytes todedifferentiated tumour cells(HE400×) werealso identified. (f): Nuclear with highmitotic activity (arrows) (HE100×). (c): Some cells present ahigher degree of pleomorphismand hyperchromasia (HE400×). (d)and (e): The (HE 50×).(b): This tumour proliferation ischaracterised by being formed by nestsor fascicles of spindle cells withareas of greater cellular atypia and Figure 3.Microscopy. (a): In theupper right normalrenal parenchyma with someglomeruliandtowards thelower left aspindle cell proliferation (asterisks) necrotic areas in30%, myxoid areas in30%andhaemorrhagicareas in10%, whichinfiltrates sinus, renalcapsuleandperineal fat. Figure 2.Macroscopy. Left kidney withthepresence of a23.0×12.010.0cmtumour, solidinappearance, soft consistency, light brown in colour with 3

Case Study ecancer 2021, 15:1173; www.ecancer.org; DOI:https://doi.org/10.3332/ecancer.2021.1173 tases. However, itsbenefit isminimalandnot many studieshave beenundertaken. Perez et al isusedinliposarcoma of kidney tumours that cannot becompletely resected, inrecurrent diseaseor in distant metas- disease andlarge tumour size at thetimeof their initialpresentation [3,7]. urinary tractsarcomas (witha 5-year survival rate ofdue to 29%–39%), thehighproportion of patients who manifest with denovo metastatic [3, 12].Surgical resection freewith margins provides ahighprobability of cure, which differs from thepoor prognosis ingeneral for genito- testsand radiology at imaging regular intervals. Ithas beenreported that postoperative hasnoimpactonrecurrence rate commonbeen themost treatment option is ensuedbywhich continuous andregular follow-up with clinicalassessment, laboratory blood appropriatewith tumour-free resectionmargins (complete resection of tumour andany invaded organ with negative margins) has (chemotherapy or radiotherapy)management inthe of primary liposarcoma of thekidney discordantwith results. In general, anoperated The mainmanagementof primary liposarcomaof kidney the isradical nephrectomy. Few caseshave reported theuseof adjuvant treatment usually low or absent andhistologically smooth with conspicuous andalsodonot lipoblasts, express MDM2 sarcomacomponent and donot express MDM2. Another oneis well-differentiated liposarcoma, however mitotic activity inthesetumours is content), dedifferentiatedsarcoma, pleomorphic liposarcoma pleomorphic andmyxoid liposarcoma, allof which lackthedifferentiated lipo- The differential diagnosesof primary liposarcoma of thekidney include: (their mainonesince both are large lesions with fat is alsorare andthere was noinvolvement of renal capsulenoted by us. fatty thrombusin the vena cava [14]. The histologicalfoundtype in our case which was acaseof undifferentiated liposarcoma of thekidney are very uncommon.Ciccarello et al (mostfrequent site of presentation) or therenal sinus[12].Primary tumours of therenal capsuleare infrequent, andliposarcomas onthissite and proteinand HMB-45 S100 and cyclin-dependent2 (MDM2) type kinase4(CDK4) The immunohistochemistry of studies renal liposarcomatend to show tumour cells thatexhibit positive stainingfor murinedouble-minute decreased survival [19]. does not generally show prognostic significance; however, inretroperitoneal tumours, high-grade morphology hasbeenassociated with in conjunction with a well-differentiated liposarcoma component. The amount andhistological grade of theundifferentiated component of undifferentiated liposarcomas of thekidney, they tend to benon-lipogenic liposarcoma of varying histological grade that often occur the kidney, those of pure round cells and undifferentiated types of liposarcoma of the kidney tend to metastasise widely [5]. In the case have ahighlocalrecurrence rate and15%rate of distant , with a5-year mortality of 30%, whereas pleomorphicliposarcoma of the well-differentiated (25%),pleomorphic(10%),undifferentiated andround cells (<5%). Well-differentiated liposarcomas of thekidney cell liposarcoma, undifferentiated liposarcoma andpleomorphicliposarcoma. The myxoid typeisthemostfrequent (60%),followed by There are five histological sub-typesof liposarcoma of thekidney including: well-differentiated liposarcoma, myxoid liposarcoma, round staining studiesof thekidney tumour isthemeansby which adefinitive diagnosisof primary liposarcoma of thekidney isdefinitely diagnosed. with contrast. Utilisation of pathology examination includingmicroscopy examination of thekidney tumour supported by immunohistochemistry does show hypo- or hyper-echoic lesion within thekidney, CT scanof kidney with contrast andmagnetic resonance imagingscanof renal tract Radiology imagingstudiesthat have beenutilisedfor theevaluation of liposarcoma of thekidney doinclude:ultrasound scanof renal tract which quent haematogenous metastases have beenreported lymphnodesandliver inthelungs, [3]. reported symptomspain, abdominalmass(most hadincluded: frequent), weight lossandhaematuria (rare presentation) [10]. The mostfre- Most casesof published liposarcoma of thekidney had reported well differentiated tumours, with anaverage tumour size of 5cmandthe in women incomparison with men have ranged between 36 years and 86 years and liposarcomas of the kidney have been stated to be three times more frequently encountered of0.8% allrenalofand 2%–3% neoplasms malignantkidney tumours. Liposarcomas of thekidney have beenreported inpatients whose ages of 119 reported cases of kidney sarcomas [5, 6]. Liposarcomas of the kidney are part of sarcomas of the genitourinary tract which represent Renal liposarcomas are extremely rare solitarydescribed incaser [20, 21].Ithas beenstated thatrenalmost liposarcomas usually tend to bederived from therenal capsule [7, 8]. [13] reported 18casesof liposarcoma with renal capsuleinvolvement, includingonecasecontaining a [11, 20].In addition,they exhibit negative stainingfor pancytokeratin, EMA,desmin [15] reported that chemotherapy agents [9, 22]. 4

Case Study ecancer 2021, 15:1173; www.ecancer.org; DOI:https://doi.org/10.3332/ecancer.2021.1173 diagnosis. Dr CMorante was involved infinalediting. involved inreference hunting andfinalediting.Dr Bravo Mand Huanca L were concerned with the histopathologic andimmunohistochemical P Rioja, G Valenciaand Centurion-Rodriguez were involved inmanuscriptpreparation, reference hunting andfinalediting. ZMorante was Authors’ contributions No funding was received for this work. Funding statement The authors have noconflicts of interest to declare. Conflicts of interest to encourage prospective andrandomised clinicaltrialsthat are focused ontreatment of thetumour. Becauseprimary liposarcomaof thekidney isanextremely rare disease,data related to thetumour islimited; therefore, itis very important the typeof surgery that hasbeenundertaken. neoadjuvantnor adjuvant the setting. Prognostic factors of primary liposarcoma of thekidney are related to thetumour characteristics and primary liposarcomaof kidney the isradicalnephrectomy. Chemotherapy andradiotherapy have not shown survival benefit inneither the of primary liposarcomaof kidney the manifestations andthe of thediseasetend to benon-specificsymptoms. The treatment of choice of Renal liposarcomas are extremely rare tumours that develop from mesenchymal tissue. Their asymptomatic onset doeslimitthediagnosis Conclusions rence rates are betweenand 85% 20% the histologicalthe anatomical type, location of thetumour andcompleteness of resection of thetumour [17,18]. The reported localrecur It hasbeeniterated that themostimportant prognosticfactors for thispathology include:thedegree of differentiation, thetumour size, was 45months (95%CI,36–66months) [16]. line chemotherapy (most often with gemcitabine/docetaxel at 56%)and12%received third line. The mediansurvival from diagnosis regimens. Chemotherapy had a response rate of 24% and a clinical benefit rate of 44%. In addition, 50% of patients received second- chemotherapy (88%), with doxorubicin andifosfamide beingthe mostcommon first-line regimen, with amedianof four cycles inall found in40%of casesanddiseaseprogression was found in40%of cases.Most patients received anthracycline-based combination scan andResponse Evaluation Criteria inSolid Tumours (RECIST): partialresponse was found in20%of thecases,stabledisease was the medianoverall survival from thestartof chemotherapy was 29months (CI: 95%,24–40). The response rates were assessedby CT sectable/metastatic or recurrent disease(n receiving neoadjuvant chemotherapy for localised or locally advanced disease(n et al differences insurvival (69months mediansurvival for truncal versus 78months for retroperitoneal liposarcomas, p such asdoxorubicin andifosfamide demonstrated activity onboth retroperitoneal andtruncalliposarcoma buthave not demonstrated reported upto 13 years after initialsurgery [5]. survival rate of 15%, a mediansurvivalwith of 28months8 to but 10months inrecurrence or metastatic disease.Recurrences have been [16] performed astudy inpatients with retroperitoneal dedifferentiated liposarcoma (n [15]. Wang =51). With regard to theresult of thestudy, Livingston et al et al [14] documented 1-year survival rate of 86%,3-year survival rate of 41%and5-year =31)andanother receiving chemotherapy for unre- =82) which was dividedintwo groups: one [16] reported thefollowing: =0.668).Livingston 5 -

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