{"orpha":424058,"name":"Intraductal papillary mucinous carcinoma of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["IPMN","Pancreatic intraductal papillary mucinous carcinoma"],"definition":"Intraductal papillary mucinous carcinoma of pancreas is a rare epithelial tumor of pancreas characterized by malignant, mucin-producing cystic mass, originating from the pancreatic ductal system, associated with local invasion and metastatic spread, composed of mucin-producing, columnar epithelial cells covering the dilated pancreatic ducts with a papillary structure. The presenting symptoms are non-specific and include abdominal pain, pancreatitis, steatorrhea, jaundice and diabetes. Many patients are asymptomatic at the time of diagnosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424058/","orphanet_url":"https://www.orpha.net/en/disease/detail/424058","names":{"fr":{"name":"Carcinome intracanalaire papillaire mucineux du pancréas","synonyms":["Carcinome intracanalaire papillaire mucineux pancréatique","TIPMP","Tumeur intracanalaire papillaire mucineuse du pancréas"],"definition":"Tumeur épithéliale rare du pancréas caractérisée par une masse kystique maligne, productrice de mucine, provenant du système canalaire pancréatique, associée à une invasion locale et à une propagation métastatique, composée de cellules épithéliales cylindriques productrices de mucine recouvrant les canaux pancréatiques dilatés qui forment des lésions à structure papillaire. Les symptômes sont non spécifiques et comprennent des douleurs abdominales, une pancréatite, une stéatorrhée, un ictère et un diabète. De nombreux patients sont asymptomatiques au moment du diagnostic."},"de":{"name":"Intraduktale papilläre muzinöse Neoplasie des Pankreas","synonyms":["IPMN"]},"es":{"name":"Carcinoma intraductal papilar mucinoso de páncreas","synonyms":["Carcinoma pancreático mucinoso intraductal papilar"],"definition":"Es un tumor epitelial del páncreas poco frecuente caracterizado por una masa quística neoplásica productora de mucina, originada en el sistema ductal pancreático, asociada a invasión local y diseminación metastásica, compuesta por células epiteliales columnares productoras de mucina, que cubren los dilatados conductos pancreáticos con una estructura papilar. Los síntomas de presentación son inespecíficos e incluyen dolor abdominal, pancreatitis, esteatorrea, ictericia y diabetes. Muchos afectados son asintomáticos en el momento del diagnóstico."},"it":{"name":"Carcinoma mucinoso papillare intraduttale del pancreas","synonyms":["Carcinoma pancreatico mucinoso papillare intraduttale"],"definition":"Il carcinoma mucinoso papillare intraduttale del pancreas è un tumore epiteliale raro, caratterizzato dalla presenza di una massa cistica maligna che produce mucina, che origina dal sistema duttale del pancreas, associata a disseminazione locale e a metastasi, composta da cellule epiteliali colonnari che producono mucina e ricoprono i dotti pancreatici dilatati con una struttura papillare. I sintomi d'esordio sono aspecifici e comprendono il dolore addominale, la pancreatite, la steatorrea, l'ittero e il diabete. Molti pazienti sono asintomatici al momento della diagnosi."},"nl":{"name":"Intraductaal papillair mucineus carcinoom van pancreas","synonyms":["Intraductaal papillair mucineus carcinoom van alvleesklier","Pancreatisch intraductaal papillair mucineus carcinoom"],"definition":"Intraductaal papillair mucineus carcinoom van de pancreas is een zeldzame epitheliale tumor van de pancreas, en wordt gekarakteriseerd door een maligne, mucine-producerende, cystische massa die ontstaat in de pancreaskanalen, die is geassocieerd met locale invasie en metastatische verspreiding, en die bestaat uit mucine-producerende, cilindrische epitheelcellen die de gedilateerde pancreaskanalen bedekken met een papillaire structuur. De presenterende symptomen zijn niet-specifiek en omvatten buikpijn, pancreatitis, steatorroe, geelzucht en diabetes. Vele patiënten zijn asymptomatisch op het moment van de diagnose."},"pt":{"name":"Carcinoma mucinoso papilar intraductal do pâncreas"},"pl":{"name":"Wewnątrzprzewodowy brodawkowaty rak śluzowy trzustki","synonyms":["IPMN","Pancreatic intraductal papillary mucinous carcinoma"],"definition":"*Wewnątrzprzewodowy brodawkowaty rak śluzowy trzustki jest rzadkim guzem trzustki pochodzenia nabłonkowego, który charakteryzuje się obecnością złośliwej torbielowatej masy produkującej śluz, pochodzący z z przewodów trzustkowych. Cechuje się miejscową inwazją i szerzy się w postaci przerzutów, zbudowanych z wytwarzających mucynę, walcowatych komórek nabłonka pokrywającego poszerzone przewody trzustkowe, które tworzą zmiany o strukturze brodawkowatej. Objawy choroby są niespecyficzne i obejmują bóle brzucha, zapalenie trzustki, stolce tłuszczowe, żółtaczkę i cukrzycę. Wielu pacjentów nie ma żadnych objawów w momencie rozpoznania."},"cs":{"name":"Intraduktální papilární mucinózní karcinom pankreatu","synonyms":["Pankreatický intraduktální papilární mucinózní karcinom"]},"uk":{"name":"Внутрішньопротокова папілярна муцинозна карцинома підшлункової залози","synonyms":["IPMN","Внутрішньопротокова папілярна муцинозна карцинома підшлункової залози"],"definition":"Intraductal papillary mucinous carcinoma of pancreas is a rare epithelial tumor of pancreas characterized by malignant, mucin-producing cystic mass, originating from the pancreatic ductal system, associated with local invasion and metastatic spread, composed of mucin-producing, columnar epithelial cells covering the dilated pancreatic ducts with a papillary structure. The presenting symptoms are non-specific and include abdominal pain, pancreatitis, steatorrhea, jaundice and diabetes. Many patients are asymptomatic at the time of diagnosis."},"zh":{"name":"胰管内乳头状黏液性癌","synonyms":["胰管内乳头状黏液性癌"],"definition":"胰腺导管内乳头状黏液癌是一种罕见的胰腺上皮肿瘤，其特征是恶性的、产生黏液的囊性肿块，起源于胰腺导管系统，伴有局部浸润和转移特性。扩张的胰管呈乳头状结构且由可产生黏蛋白的柱状上皮组成。临床症状是非特异性的，包括腹痛，胰腺炎，脂肪泻，黄疸和糖尿病。许多患者在诊断时无症状。"},"tr":{"name":"Pankreasın intraduktal papiller müsinöz karsinomu","synonyms":["IPMN","Pankreatik intraduktal papiller müsinöz karsinom"]}},"xrefs":[{"source":"ICD-10","code":"C25.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C10.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2086328209"},{"source":"MONDO","code":"0004285","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1335304","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10073365","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":0.018,"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":0.028,"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":0.016,"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":0.001,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.011,"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":0.017,"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":0.028,"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":0.031,"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":0.048,"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":0.006,"area":"Ireland","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":"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":0.041,"area":"Norway","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":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}