{"orpha":424053,"name":"Mucinous cystadenocarcinoma of the pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Pancreatic mucinous cystadenocarcinoma"],"definition":"A rare, epithelial tumor of the pancreas characterized, histologically, by columnar, mucin-producing epithelium associated with ovarian-type subepithelial stroma, which does not communicate with the pancreatic ductal system, most frequently localized to the body or tail of the pancreas. Clinically, small tumors (<3 cm) are usually asymptomatic, while large tumors typically present obstructive jaundice, a palpable abdominal mass, and may associate portal hypertension, hemobilia and diabetes mellitus.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424053/","orphanet_url":"https://www.orpha.net/en/disease/detail/424053","names":{"fr":{"name":"Cystadénocarcinome mucineux du pancréas","synonyms":["Cystadénocarcinome mucineux pancréatique"],"definition":"Tumeur épithéliale rare du pancréas caractérisée, sur le plan histologique, par un épithélium cylindrique produisant de la mucine, associé à un stroma sous-épithélial de type ovarien qui ne communique pas avec le canal pancréatique, siégeant le plus fréquemment sur le corps ou la queue du pancréas. Les tumeurs de petite taille (< 3 cm) sont généralement asymptomatiques, alors que les patients atteints de plus grandes tumeurs présentent généralement une jaunisse obstructive et une masse abdominale détectable à la palpation, potentiellement associées à une hypertension portale, une hémobilie et un diabète sucré."},"de":{"name":"Muzinöses Zystadenokarzinom des Pankreas","synonyms":["MCN","Muzinös-zystische Neoplasie des Pankreas"],"definition":"Ein seltener epithelialer Tumor der Bauchspeicheldrüse, der histologisch durch ein säulenförmiges, Muzin produzierendes Epithel gekennzeichnet ist, das mit einem subepithelialen Stroma vom Ovarialtyp assoziiert ist und nicht mit dem Gangsystem der Bauchspeicheldrüse kommuniziert. Der Tumor ist meist im Körper oder im Schwanz der Bauchspeicheldrüse lokalisiert. Klinisch gesehen sind kleine Tumore (<3 cm) in der Regel asymptomatisch, während große Tumore typischerweise eine tastbare abdominale Masse aufweisen und mit einer obstruktiven Gelbsucht einhergehen. Weitere Manifestationen umfassen portale Hypertension, Hämobilität und Diabetes mellitus."},"es":{"name":"Cistoadenocarcinoma mucinoso de páncreas","synonyms":["Cistoadenocarcinoma pancreático mucinoso"],"definition":"Es un tumor pancreático epitelial poco frecuente caracterizado histológicamente por un epitelio columnar productor de mucina asociado a estroma subepitelial de tipo ovárico, sin comunicación con el sistema ductal pancreático y localizado con mayor frecuencia en el cuerpo o en la cola del páncreas. Clínicamente, los tumores pequeños (<3 cm) suelen ser asintomáticos, mientras que los tumores grandes presentan típicamente ictericia obstructiva, una masa abdominal palpable y pueden asociar hipertensión portal, hemobilia y diabetes mellitus."},"it":{"name":"Cistadenocarcinoma mucinoso del pancreas","synonyms":["Cistadenocarcinoma mucinoso pancreatico"],"definition":"È una neoplasia epiteliale rara del pancreas, caratterizzata, dal punto di vista istologico, da epitelio colonnare che produce mucina, associato a stroma subepiteliale di tipo ovarico, che non comunica con il sistema duttale del pancreas. Di solito si localizza nel corpo o nella coda del pancreas. Dal punto di vista clinico, i tumori di piccole dimensioni (< 3 cm) di solito sono asintomatici, mentre quelli più grandi in genere esordiscono con ittero ostruttivo e con la presenza di una massa addominale palpabile, e possono associarsi ad ipertensione portale, emobilia e diabete mellito."},"nl":{"name":"Mucineus cystadenocarcinoom van pancreas","synonyms":["Mucineus cystadenocarcinoom van alvleesklier","Pancreatisch mucineus cystadenocarcinoom"],"definition":"Een zeldzame epitheeltumor van de pancreas histologisch gekarakteriseerd door columnair, mucine-producerend epitheel, geassocieerd met ovarieel subepitheliaal stroma dat niet communiceert met het kanaalsysteem van de pancreas en meestal gelokaliseerd is in het lichaam of de staart van de pancreas. Kleine tumoren (<3 cm) zijn meestal klinisch asymptomatisch, terwijl grote tumoren zich doorgaans presenteren met obstructieve geelzucht en een tastbare abdominale massa, en mogelijk portale hypertensie, hemobilie en diabetes mellitus associëren."},"pt":{"name":"Cistoadenocarcinoma mucinoso do pâncreas"},"pl":{"name":"Torbielogruczolakorak śluzowy trzustki","synonyms":["Pancreatic mucinous cystadenocarcinoma"]},"cs":{"name":"Mucinózní cystadenokarcinom pankreatu","synonyms":["Pankreatický mucinózní cystadenokarcinom"]},"uk":{"name":"Муцинозна цистаденокарцинома підшлункової залози","synonyms":["Муцинозна цистаденокарцинома підшлункової залози"],"definition":"A rare, epithelial tumor of the pancreas characterized, histologically, by columnar, mucin-producing epithelium associated with ovarian-type subepithelial stroma, which does not communicate with the pancreatic ductal system, most frequently localized to the body or tail of the pancreas. Clinically, small tumors (<3 cm) are usually asymptomatic, while large tumors typically present obstructive jaundice, a palpable abdominal mass, and may associate portal hypertension, hemobilia and diabetes mellitus."},"zh":{"name":"胰腺粘液性囊腺癌","synonyms":["胰腺粘液性囊腺癌"],"definition":"一种罕见的胰腺上皮性肿瘤，在组织学上的特征是与卵巢型上皮下基质相关的 柱状的，产生粘蛋白的上皮细胞，其不与胰腺导管系统相通，最常见于胰腺体或尾。临床上，小肿瘤（ sup 3 cm）通常无症状，而大肿瘤通常表现为梗阻性黄疸，可触及的腹部肿块，并可能伴有门脉高压症，肝硬化和糖尿病。"},"tr":{"name":"Pankreasın müsinöz kistadenokarsinomu","synonyms":["Pankreatik müsinöz kistadenokarsinomu"]}},"xrefs":[{"source":"ICD-10","code":"C25.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C25.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C25.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C25.7","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C25.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C10.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"341848547"},{"source":"MONDO","code":"0018523","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18523","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2063873","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":217074,"name":"Rare carcinoma of pancreas","classifications":[152,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.01,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.006,"area":"Belgium","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.002,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.002,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.012,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.003,"area":"France","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.012,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.019,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.004,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.024,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.006,"area":"Switzerland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.019,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.004,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}