{"orpha":319287,"name":"Multilocular cystic renal neoplasm of low malignant potential","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["MCRCC","Multilocular clear cell adenocarcinoma","Multilocular clear cell carcinoma","Multilocular clear cell renal cell adenocarcinoma","Multilocular clear cell renal cell carcinoma","Multilocular cystic renal cell adenocarcinoma","Multilocular cystic renal cell carcinoma"],"definition":"Multilocular cystic renal neoplasm of low malignant potential is a rare subtype of clear cell renal cell carcinoma with distinct pathological features of cysts lined by occasionally flattened cuboidal clear cells and septa containing aggregates of epithelial cells with clear cytoplasm, and excellent prognosis. The tumor usually presents as an asymptomatic, unilateral, solitary lesion, macroscopically consisting of numerous, fluid-filled, septated cysts of variable size. Rarely, the symptoms typically associated with renal tumors (flank pain, hematuria, palpable mass) may be present.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-319287/","orphanet_url":"https://www.orpha.net/en/disease/detail/319287","names":{"fr":{"name":"Carcinome rénal kystique multiloculaire","synonyms":["Carcinome à cellules rénales kystique multiloculaire","MCRCC"],"definition":"Un sous-type rare de carcinome rénal à cellules claires, dont les caractéristiques pathologiques distinctes sont des kystes bordés occasionnellement par des cellules claires cuboïdes aplaties et par des septums contenant des agrégats de cellules épithéliales à cytoplasme clair. Le pronostic est excellent. La tumeur se manifeste généralement par une lésion solitaire, unilatérale et asymptomatique, caractérisée sur le plan macroscopique par de nombreux kystes septaux de taille variable et remplis de liquide. Des signes liés à des tumeurs rénales (douleur au flanc, hématurie, masse palpable) peuvent être présents dans de rares cas."},"de":{"name":"Multilokuläre zystische Neoplasie der Niere von geringem malignen Potential","synonyms":["Adenokarzinom, klarzelliges multilokuläres","Karzinom, klarzelliges multilokuläres","MCRCC","Multilokuläres klarzelliges Adenokarzinom der Niere","Nierenzellkarzinom, klarzelliges multilokuläres","Nierenzellkarzinom, multilokuläres zystisches","Adenokarzinom der Niere, multilokuläres klarzelliges","Adenokarzinom der Niere, multilokuläres zystisches"],"definition":"Ein seltener Subtyp des klarzelligen Nierenzellkarzinoms mit einer ausgezeichneten Prognose. Es präsentiert sich mit charakteristischen pathologischen Merkmalen und besteht nahezu ausschließlich aus Zysten, die von gelegentlich abgeflachten quaderförmigen klaren Zellen und Septen ausgekleidet sind, die Aggregate von Epithelzellen mit klarem Zytoplasma enthalten. Der Tumor präsentiert sich in der Regel als asymptomatische, unilaterale, solitäre Läsion, die makroskopisch aus zahlreichen, flüssigkeitsgefüllten, septierten Zysten von unterschiedlicher Größe besteht. Selten können die typischerweise mit Nierentumoren assoziierten Symptome (Flankenschmerzen, Hämaturie, tastbare Masse) vorhanden sein."},"es":{"name":"Neoplasia renal quística multilocular de bajo potencial maligno","synonyms":["Adenocarcinoma multilocular de células claras","Adenocarcinoma renal multilocular de células claras","Carcinoma multilocular de células renales de células claras","Carcinoma quistico multilocular de células renales","MCRCC"],"definition":"La neoplasia renal quística multilocular de bajo potencial maligno es un subtipo poco frecuente de carcinoma renal de células claras con características patológicas distintivas consistentes en quistes revestidos por células claras cuboidales ocasionalmente aplanadas y tabiques que contienen agregados de células epiteliales con citoplasma claro, y de pronóstico excelente. Generalmente el tumor se presenta como una lesión aislada, unilateral y asintomática, consistente macroscópicamente en numerosos quistes septados de tamaño variable llenos de líquido. Excepcionalmente pueden presentarse síntomas característicos de los tumores renales (dolor en flanco, hematuria, masa palpable)."},"it":{"name":"Neoplasia renale cistica multiloculare a basso potenziale di malignità","definition":"La neoplasia renale cistica multiloculare a basso potenziale di malignità è un sottotipo raro del carcinoma renale a cellule chiare. È caratterizzato da cisti rivestite occasionalmente da cellule chiare cuboidi appiattite e da setti contenenti aggregati di cellule epiteliali con citoplasma chiaro. La prognosi è molto favorevole. Di solito il tumore esordisce come lesione isolata, monolaterale asintomatica, costituita, dal punto di vista macroscopico, da numerose cisti settate contenenti liquido, di dimensioni variabili. Di rado i pazienti presentano i sintomi caratteristici dei tumori renali (dolore al fianco, ematuria e presenza di una massa palpabile)."},"nl":{"name":"Multiloculair cystisch neoplasma van nier met laag maligne potentieel","synonyms":["MCRCC","Multiloculair cystisch nierceladenocarcinoom","Multiloculair cystisch niercelcarcinoom","Multiloculair heldercellig adenocarcinoom","Multiloculair heldercellig carcinoom","Multiloculair heldercellig nierceladenocarcinoom","Multiloculair heldercellig niercelcarcinoom"],"definition":"Multiloculair cystisch neoplasma van de nier met laag maligne potentieel is een zeldzaam subtype van heldercellig niercelcarcinoom, met als specifieke pathologische kenmerken cysten bekleed met occasioneel afgevlakte cuboïdale heldere cellen en septa die aggregaten bevatten van epitheelcellen met helder cytoplasma, alsook een zeer goede prognose. De tumor vertoont zich meestal als een asymptomatisch, unilateraal, solitair letsel, dat macroscopisch bestaat uit vele, met vocht gevulde, gesepteerde cysten met een variabele omvang. In zeldzame gevallen kunnen de symptomen die doorgaans geassocieerd zijn met niertumoren (pijn in de flanken, hematurie, tastbare massa) aanwezig zijn."},"pt":{"name":"Carcinoma de células renais multilocular quístico"},"pl":{"name":"Wieloogniskowy torbielowaty jasnokomórkowy nowotwór nerki o niskim stopniu złośliwości","synonyms":["MCRCC","Wieloogniskowy torbielowaty rak z komórek nerkowych","Wieloogniskowy torbielowaty gruczolakorak z komórek nerkowych"]},"cs":{"name":"Multilokulární cystický karcinom ledviny","synonyms":["Multilokulární cystický renální adenokarcinom","Multilokulární adenokarcinom světlých buněk","Multilokulární karcinom světlých buněk","Multilokulární adenokarcinom světlých renálních buněk","Multilokulární karcinom světlých renálních buněk"]},"uk":{"name":"Мультилокулярна кістозна пухлина нирки з низьким злоякісним потенціалом","synonyms":["MCRCC","Мультилокулярна світлоклітинна аденокарцинома","Мультилокулярна світлоклітинна карцинома","Мультилокулярна прозороклітинна нирковоклітинна аденокарцинома","Мультилокулярна світлоклітинна нирковоклітинна карцинома","Мультилокулярна кістозна нирковоклітинна аденокарцинома","Мультилокулярна кістозна нирковоклітинна карцинома"],"definition":"Multilocular cystic renal neoplasm of low malignant potential is a rare subtype of clear cell renal cell carcinoma with distinct pathological features of cysts lined by occasionally flattened cuboidal clear cells and septa containing aggregates of epithelial cells with clear cytoplasm, and excellent prognosis. The tumor usually presents as an asymptomatic, unilateral, solitary lesion, macroscopically consisting of numerous, fluid-filled, septated cysts of variable size. Rarely, the symptoms typically associated with renal tumors (flank pain, hematuria, palpable mass) may be present."},"zh":{"name":"低度恶性多囊肾肿瘤","synonyms":["多男性透明细胞腺癌"],"definition":"多房性囊性肾肿瘤是一种罕见的透明细胞性肾癌，其病理学特征为囊肿内有偶有扁平的立方透明细胞和胞浆透明的上皮细胞团构成的间隔，预后良好。肿瘤通常表现为无症状，单侧，孤立性病变，肉眼可见大量充满液体的大小不等的分隔囊肿。很少出现与肾肿瘤相关的症状（腰痛、血尿、可触及肿块）。"},"tr":{"name":"Düşük malignite potansiyeline sahip multiloküler kistik renal neoplazm","synonyms":["Multiloküler kistik renal hücreli karsinom","Multiloküler berrak hücreli renal hücreli karsinom","Multiloküler kistik renal hücreli adenokarsinom","Multiloküler berrak renal hücreli adenokarsinom","MCRCC","Multiloküler berrak hücreli karsinom","Multiloküler berrak hücreli adenokarsinom"]}},"xrefs":[{"source":"ICD-10","code":"C64","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0003010","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0346249","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":319276,"name":"Clear cell renal carcinoma","classifications":[188,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}