{"orpha":404514,"name":"Acquired cystic disease-associated renal cell carcinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare subtype of renal cell carcinoma, ocurring in the context of end-stage kidney disease and acquired cystic kidney disease, characterized by a usually well circumscribed, solid, multifocal, bilateral tumor with inter- or intracellular microlumen formation (leading to cribiform architecture). Tumors are often diagnosed incidentally in early stages, although complications caused by renal cysts (dull flank or abdominal pain, fever) or renal parenchymal bleeding may mask the underlying neoplastic process. Most have an indolent behavior.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-404514/","orphanet_url":"https://www.orpha.net/en/disease/detail/404514","names":{"fr":{"name":"Carcinome rénal associé à une maladie kystique acquise","definition":"Un sous-type rare de carcinome rénal survenant dans le contexte d'une insuffisance rénale terminale et d'une maladie rénale kystique acquise. Il se caractérise par une tumeur bilatérale, multifocale, solide, généralement bien circonscrite, avec la formation d'un microlumen inter- ou intracellulaire (conduisant à une architecture cribriforme). La tumeur est souvent diagnostiquée de manière fortuite dans les stades précoces. Cependant, les complications causées par les kystes rénaux (douleur sourde au flanc ou à l'abdomen, fièvre) ou un saignement du parenchyme rénal peuvent masquer le processus néoplasique sous-jacent. La tumeur a habituellement un comportement indolent."},"de":{"name":"Erworbene Nierenkrankheit mit assoziiertem Nierenzellkarzinom","definition":"Ein seltener Subtyp des Nierenzellkarzinoms, der im Rahmen einer Nierenerkrankung im Endstadium und einer erworbenen zystischen Nierenerkrankung auftritt und durch einen meist gut umschriebenen, soliden, multifokalen, bilateralen Tumor mit inter- oder intrazellulärer Mikrolumenbildung (die zu einer kribriformen Architektur führt) gekennzeichnet ist. Die Tumoren werden oft zufällig in frühen Stadien diagnostiziert, obwohl Komplikationen durch Nierenzysten (dumpfe Flanken- oder Bauchschmerzen, Fieber) oder Nierenparenchymblutungen den zugrunde liegenden neoplastischen Prozess maskieren können. Die meisten haben ein indolentes Verhalten."},"es":{"name":"Carcinoma de células renales adquirido asociado con una enfermedad quística","definition":"Es un subtipo de carcinoma de células renales, poco frecuente, que se presenta en el contexto de enfermedad renal terminal y de enfermedad renal quística adquirida, y que se caracteriza por un tumor generalmente bien circunscrito, sólido, multifocal, bilateral, con formación de microlumen inter- o intracelular (que conduce a un patrón cribiforme). A menudo, los tumores se diagnostican de manera incidental en etapas tempranas, aunque las complicaciones causadas por quistes renales (dolor abdominal o en flanco sordo, fiebre) o sangrado del parénquima renal pueden enmascarar el proceso neoplásico subyacente. La mayoría presenta un comportamiento indolente."},"it":{"name":"Malattia cistica acquisita associata al carcinoma delle cellule renali","definition":"La malattia cistica acquisita associata al carcinoma delle cellule renali è un sottotipo raro di carcinoma renale che esordisce nel quadro di una malattia renale terminale e di una malattia cistica acquisita. È caratterizzato da un tumore bilaterale, di solito ben circoscritto, solido e multifocale, con formazione di microlumi inter- o intra-cellulari (che esitano in un'architettura cribriforme). Spesso i tumori vengono diagnosticati casualmente e precocemente. Tuttavia, le complicanze delle cisti renali (dolore sordo al fianco o dolore addominale e febbre) oppure l'emorragia del parenchima renale possono mascherare la neoplasia. La maggior parte di questi tumori è silente."},"nl":{"name":"Niercelcarcinoom geassocieerd met verworven cystische ziekte","definition":"Een zeldzaam subtype van niercelcarcinoom, dat voorkomt in de context van terminaal nierfalen en verworven cystische nierziekte, en dat gekarakteriseerd wordt door een doorgaans goed begrensde, solide, multifocale, bilaterale tumor met vorming van inter- of intracellulair microlumen (wat leidt tot een cribriforme architectuur). Tumoren worden vaak toevallig gediagnosticeerd in de vroege stadia, hoewel complicaties veroorzaakt door niercysten (doffe pijn in de flank of buik, koorts) of bloeding van nierparenchym het onderliggende neoplastische proces kunnen maskeren. De meeste tumoren vertonen een indolent gedrag."},"pt":{"name":"Carcinoma de células renais associado a doença renal quística adquirida"},"pl":{"name":"Nabyta choroba torbielowata nerek towarzysząca rakowi nerkowokomórkowemu"},"cs":{"name":"Získané onemocnění ledvin asociované s renálním karcinomem"},"uk":{"name":"Набута нирковоклітинна карцинома, асоційована з кістозною хворобою","definition":"A rare subtype of renal cell carcinoma, ocurring in the context of end-stage kidney disease and acquired cystic kidney disease, characterized by a usually well circumscribed, solid, multifocal, bilateral tumor with inter- or intracellular microlumen formation (leading to cribiform architecture). Tumors are often diagnosed incidentally in early stages, although complications caused by renal cysts (dull flank or abdominal pain, fever) or renal parenchymal bleeding may mask the underlying neoplastic process. Most have an indolent behavior."},"zh":{"name":"获得性囊性疾病相关性肾细胞癌","definition":"肾细胞癌的一种罕见类型，在终末期肾病和获得性囊性肾病的基础上产生，主要表现为边界清晰的 实体的，多灶的，双侧的，细胞内或细胞外微腔形成（导致筛状结构）。肿瘤通常在早期意外诊断。尽管肾脏疾病的病程有可能会被肾囊肿导致的并发症（腹部钝痛或疼痛、发热）或肾实质出血给掩盖。多数人表现为懒惰行为。"},"tr":{"name":"Edinsel kistik hastalık-ilişkili renal hücreli karsinom"}},"xrefs":[{"source":"ICD-10","code":"C64","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018449","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707179","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":217071,"name":"Renal cell carcinoma","classifications":[188,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[],"phenotypes":null}