{"orpha":247203,"name":"Collecting duct carcinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["BDC","Bellini carcinoma","Bellini duct carcinoma","CDC"],"definition":"Collecting duct carcinoma is a rare, aggressive subtype of renal cell carcinoma, which originates from the epithelium of the distal collecting ducts, and usually manifests with hematuria, flank pain, palpable abdominal mass or nonspecific symptoms, such as fatigue, weight loss or fever. Patients are often asymptomatic for long periods of time and therefore, disease is often locally advanced or metastatic at the time of diagnosis. In cases with metastatic spread, bone pain, cough, dyspnea, pneumonia or neurological compromise may be associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-247203/","orphanet_url":"https://www.orpha.net/en/disease/detail/247203","names":{"fr":{"name":"Carcinome des tubes collecteurs","synonyms":["BDC","CDC","Carcinome de Bellini","Carcinome des tubes de Bellini"],"definition":"Le carcinome des tubes collecteurs est un sous-type rare et agressif de carcinome des cellules rénales issu de l'épithélium des tubes collecteurs distaux. Il se manifeste habituellement par une hématurie, une douleur au flanc, une masse abdominale palpable, ou par des signes non spécifiques tels qu'une fatigue, une perte de poids ou de la fièvre. Les patients sont souvent asymptomatiques pendant longtemps. De ce fait, la maladie est souvent avancée au niveau local, ou est métastatique au moment du diagnostic. Une douleur osseuse, une toux, une dyspnée, une pneumonie ou une atteinte neurologique peuvent être associés aux cas métastatiques."},"de":{"name":"Sammelgangkarzinom","synonyms":["DBK","Ductus-Bellini-Karzinom"],"definition":"Das Ductus-Bellini-Karzinom ist ein seltener, aggressiver Subtyp des Nierenzellkarzinoms, der vom Epithel der distalen Sammelgänge ausgeht und sich in der Regel mit Hämaturie, Flankenschmerzen, tastbarer abdomineller Masse oder unspezifischen Symptomen wie Müdigkeit, Gewichtsverlust oder Fieber manifestiert. Die Patienten sind oft lange Zeit asymptomatisch, daher ist die Erkrankung zum Zeitpunkt der Diagnose oft lokal fortgeschritten oder metastasiert. In Fällen mit metastatischer Ausbreitung können Knochenschmerzen, Husten, Dyspnoe, Lungenentzündung oder neurologische Beeinträchtigungen auftreten."},"es":{"name":"Carcinoma de los túbulos colectores","synonyms":["BDC","CDC","Carcinoma de Bellini","Carcinoma de los conductos de Bellini"],"definition":"El carcinoma de los túbulos colectores es un subtipo agresivo de carcinoma de células renales poco frecuente que se origina en el epitelio de los túbulos colectores distales y que, por lo general, se manifiesta con hematuria, dolor en el costado, masa abdominal palpable o síntomas inespecíficos, tales como fatiga, pérdida de peso o fiebre. Con frecuencia, los afectados son asintomáticos durante largos períodos de tiempo y, por lo tanto, la enfermedad es localmente avanzada o metastásica en el momento del diagnóstico. En casos con diseminación metastásica puede presentarse dolor de huesos, tos, disnea, neumonía o compromiso neurológico asociados."},"it":{"name":"Carcinoma dei dotti collettori","synonyms":["Carcinoma dei dotti di Bellini","Carcinoma di Bellini","BDC","CDC"],"definition":"Il carcinoma del dotto collettore è un sottotipo raro e aggressivo di carcinoma delle cellule renali, che origina nell'epitelio dei dotti collettori distali e di solito esordisce con ematuria, dolore al fianco e la presenza di una massa addominale palpabile, oppure con sintomi aspecifici come l'affaticamento, la perdita di peso o la febbre. Spesso i pazienti sono a lungo asintomatici e, per questo, al momento della diagnosi la malattia si trova spesso uno stadio avanzato a livello locale o addirittura è già metastatizzata. In presenza di metastasi, possono coesistere dolore osseo, tosse, dispnea, polmonite, compromissione neurologica."},"nl":{"name":"Verzamelkanaalcarcinoom","synonyms":["BDC","Bellinicarcinoom","Bellinikanaalcarcinoom","CDC"],"definition":"Verzamelkanaalcarcinoom is een zeldzaam, agressief subtype van niercelcarcinoom dat voortkomt uit het epitheel van de distale verzamelkanaaltjes, en dat zich doorgaans manifesteert met hematurie, pijn aan de zij, een tastbare abdominale massa, of niet-specifieke symptomen zoals vermoeidheid, gewichtsverlies of koorts. Patiënten zijn vaak asymptomatisch gedurende lange periodes, waardoor de ziekte vaak lokaal gevorderd of uitgezaaid is op het moment van de diagnose. Bij gevallen met uitzaaiingen kunnen botpijn, hoest, dyspnoe, pneumonie of neurologische schade geassocieerd zijn."},"pt":{"name":"Carcinoma dos ductos coletores"},"pl":{"name":"Rak kanalików zbiorczych nerki","synonyms":["BDC","CDC","Rak Belliniego","Rak kanalików Belliniego"]},"cs":{"name":"Karcinom ze sběrných kanálků","synonyms":["BCD","Belliniho karcinom","CDC","Karcinom ze sběrných Belliniho kanálků"]},"uk":{"name":"Збірна протокова карцинома","synonyms":["BDC","Карцинома Белліні","Карцинома протоки Белліні","CDC"],"definition":"Collecting duct carcinoma is a rare, aggressive subtype of renal cell carcinoma, which originates from the epithelium of the distal collecting ducts, and usually manifests with hematuria, flank pain, palpable abdominal mass or nonspecific symptoms, such as fatigue, weight loss or fever. Patients are often asymptomatic for long periods of time and therefore, disease is often locally advanced or metastatic at the time of diagnosis. In cases with metastatic spread, bone pain, cough, dyspnea, pneumonia or neurological compromise may be associated."},"zh":{"name":"集合管癌","synonyms":["Bellini管癌"],"definition":"肾脏集合管癌是肾细胞癌的一种罕见的侵袭性亚型，起源于远端集合管上皮，通常表现为血尿、腰痛、腹部肿块或非特异性症状，如疲劳、体重减轻或发烧。患者通常长期无症状表现，因此，在诊断时，疾病通常已发生局部进展或转移。转移性扩散的病例可能与骨痛、咳嗽、呼吸困难、肺炎或神经系统损害有关。"},"tr":{"name":"Toplayıcı duktus karsinomu","synonyms":["BDC","HKM","Bellini karsinomu","Bellini kanal karsinomu"]}},"xrefs":[{"source":"ICD-10","code":"C64","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0005220","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1266044","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9573","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"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}