{"orpha":97366,"name":"Multiloculated renal cyst","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Multilocular cyst of the kidney","Multilocular renal cyst"],"definition":"A rare benign renal tumor characterized by a typically unilateral, solitary, multiloculated cystic mass consisting of small, non-communicating cysts with flat, cuboidal, or hobnail epithelial lining, separated by fibrous septa which may have an ovarian stroma-like appearance or be paucicellular. The tumor is surrounded by a thick fibrous capsule and does not contain solid areas or necrosis. Patients may be asymptomatic or present with a palpable abdominal mass and/or abdominal or flank pain. Age distribution is bimodal, the typical age of onset being either below five or between 40 and 70 years of age.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97366/","orphanet_url":"https://www.orpha.net/en/disease/detail/97366","names":{"fr":{"name":"Kyste multiloculaire du rein","synonyms":["Kyste rénal multiloculaire","Néphrome kystique"],"definition":"Tumeur rénale bénigne rare caractérisée par une masse kystique multiloculaire, isolée et généralement unilatérale composée de petits kystes non communicants revêtus de cellules épithéliales aplaties, cuboïdes ou en clous de tapissier, séparés par des septa fibreux pouvant ressembler au stroma ovarien ou être paucicellulaires. La tumeur est entourée d'une épaisse capsule fibreuse et ne renferme aucune zone solide ou nécrosée. Les patients peuvent être asymptomatiques ou présenter une masse abdominale à la palpation et/ou des douleurs au flanc. La distribution par âge est bimodale, la maladie se déclarant soit chez l'enfant de moins de 5 ans soit chez l'adulte âgé de 40 à 70 ans."},"de":{"name":"Nierenzysten, septierte","synonyms":["Septierte Zysten der Niere"],"definition":"Ein seltener gutartiger Nierentumor, der durch eine typischerweise einseitige, solitäre, multilokuläre zystische Gewebeverdichtung gekennzeichnet ist, die aus kleinen, nicht kommunizierenden Zysten mit flacher, quaderförmiger oder hobnagelförmiger Epithelauskleidung besteht, die durch fibröse Septen voneinander getrennt sind und ein ovariales stromaähnliches Aussehen haben oder paucicellulär sein können. Der Tumor ist von einer dicken fibrösen Kapsel umgeben und enthält keine festen Bereiche oder Nekrosen. Die Patientinnen können asymptomatisch sein oder sich mit einer tastbaren Gewebeverdichtung und/oder Bauch- oder Flankenschmerzen vorstellen. Die Altersverteilung ist bimodal, wobei das typische Erkrankungsalter entweder unter fünf oder zwischen 40 und 70 Jahren liegt."},"es":{"name":"Quiste renal múltiple","synonyms":["Quiste multilocular del riñón","Quiste renal multilocular"],"definition":"Es un tumor renal benigno poco frecuente caracterizado por una masa típicamente quística multiloculada, aislada y unilateral, compuesta por pequeños quistes no comunicantes con un revestimiento epitelial plano, cuboidal o en forma de tachuela, separados por tabiques fibrosos, que pueden presentar un aspecto similar al del estroma ovárico o ser paucicelulares. El tumor está rodeado por una cápsula fibrosa gruesa y no contiene áreas sólidas ni necrosis. Los pacientes pueden permanecer asintomáticos o presentar una masa abdominal palpable y/o dolor abdominal o en flanco. La distribución por edades es bimodal, siendo la edad típica al diagnóstico inferior a los 5 años o entre los 40 y los 70 años de edad."},"it":{"name":"Cisti renale multiloculare","synonyms":["Cisti multiloculare del rene"],"definition":"Si tratta di un tumore benigno raro del rene, caratterizzato da una massa cistica in genere monolaterale, solitaria, multiloculata, costituita da piccole cisti non comunicanti con rivestimento epiteliale piatto, cuboidale o ''a chiodo'', separate da setti fibrosi che possono avere un aspetto simile allo stroma ovarico o essere paucicellulari. Il tumore è circondato da una spessa capsula fibrosa e non contiene aree solide o necrosi. I pazienti possono essere asintomatici oppure presentare una massa addominale palpabile e/o dolore addominale o al fianco. La distribuzione per età è bimodale; di solito esordisce prima dei 5 anni o tra i 40 e i 70 anni."},"nl":{"name":"Multiloculaire renale cyste","synonyms":["Multiloculaire cyste van de nier","Multiloculaire niercyste"],"definition":"Een zeldzame goedaardige tumor van nier, gekarakteriseerd door een doorgaans unilaterale, solitaire, multiloculaire cystische massa bestaande uit kleine, niet-communicerende cysten met platte, cuboïdale of kopspijker-achtige epitheliale bekleding, gescheiden door fibreuze septa die kunnen lijken op ovarieel stroma of weinig cellen bevatten. De tumor wordt omringd door een dikke fibreuze capsule en bevat geen solide delen of necrose. Patiënten kunnen asymptomatisch zijn, of zich presenteren met een palpabele abdominale massa en/of abdominale pijn of flankpijn. De leeftijdsverdeling is bimodaal, met een typische aanvangsleeftijd onder vijf jaar of tussen 40 en 70 jaar."},"pt":{"name":"Quisto renal, multiloculado"},"pl":{"name":"Wielokomorowa torbiel nerki","synonyms":["Wielokomorowa torbiel nerki","Wielokomorowa torbiel nerki"]},"cs":{"name":"Mnohočetné renální cysty","synonyms":["Mnohočetné cysty ledvin"]},"uk":{"name":"Мультилокулярна кіста нирки","synonyms":["Мультилокулярна кіста нирки","Мультилокулярна кіста нирки"],"definition":"A rare benign renal tumor characterized by a typically unilateral, solitary, multiloculated cystic mass consisting of small, non-communicating cysts with flat, cuboidal, or hobnail epithelial lining, separated by fibrous septa which may have an ovarian stroma-like appearance or be paucicellular. The tumor is surrounded by a thick fibrous capsule and does not contain solid areas or necrosis. Patients may be asymptomatic or present with a palpable abdominal mass and/or abdominal or flank pain. Age distribution is bimodal, the typical age of onset being either below five or between 40 and 70 years of age."},"zh":{"name":"多囊性肾囊肿","synonyms":["肾脏多房囊肿"]},"tr":{"name":"Multiloküle böbrek kisti","synonyms":["Multiloküler böbrek kisti","Böbreğin multiloküler kisti"]}},"xrefs":[{"source":"ICD-10","code":"D30.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019983","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0431719","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":506213,"name":"Rare disorder potentially indicated for kidney transplant","classifications":[233]},{"orpha":93619,"name":"Rare renal tumor","classifications":[188,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult","Elderly","Infancy"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}