{"orpha":424073,"name":"Serous cystadenocarcinoma of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Pancreatic serous cystadenocarcinoma"],"definition":"A very rare, malignant, epithelial tumor of the pancreas composed of cystic structures lined by glycogen-rich clear cells, associated with local invasiveness often involving the spleen, duodenum and/or stomach and metastatic spread to the liver, peritoneum and/or lymph nodes. Presenting symptoms are variable and usually non-specific and include abdominal and/or flank pain, palpable abdominal mass, upper gastrointestinal bleeding, jaundice or abnormal serum liver enzymes, vomiting, anorexia and/or weight loss.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424073/","orphanet_url":"https://www.orpha.net/en/disease/detail/424073","names":{"fr":{"name":"Cystadénocarcinome séreux du pancréas","synonyms":["Cystadénocarcinome séreux pancréatique"],"definition":"Tumeur épithéliale maligne rare du pancréas composée de structures kystiques bordées de cellules claires riches en glycogène, associée à une invasion locale (le plus souvent de la rate, du duodénum et/ou de l'estomac) et à une dissémination métastatique au foie, au péritoine et/ou aux ganglions lymphatiques. Variables et généralement non spécifiques, les symptômes initiaux comprennent douleurs abdominales et/ou douleurs au flanc, masse abdominale palpable, hémorragies digestives haute, ictère ou taux anormaux d'enzymes hépatiques sériques, vomissements, anorexie et/ou perte de poids."},"de":{"name":"Seröses Zystadenokarzinom des Pankreas","synonyms":["Pankreatisches seröses Zystadenokarzinom","SCN","Serös-zystische Neoplasien des Pankreas"]},"es":{"name":"Cistoadenocarcinoma seroso de páncreas","synonyms":["Cistoadenocarcinoma pancreático seroso"],"definition":"Es un tumor pancreático epitelial maligno muy poco frecuente compuesto por estructuras quísticas revestidas por células claras ricas en glucógeno, asociado a invasividad local que a menudo afecta al bazo, duodeno y/o estómago con diseminación metastásica al hígado, peritoneo y/o ganglios linfáticos. Los síntomas iniciales son variables y, por lo general, inespecíficos e incluyen dolor abdominal y/o en flanco, masa abdominal palpable, hemorragia digestiva alta, ictericia o alteración de las enzimas hepáticas en suero, vómitos, anorexia y/o pérdida de peso."},"it":{"name":"Cistoadenocarcinoma sieroso del pancreas","synonyms":["Cistoadenocarcinoma pancreatico sieroso"],"definition":"È una neoplasia epiteliale maligna molto rara del pancreas, composta da strutture cistiche rivestite da cellule chiare ricche di glicogeno, localmente invasiva, che spesso interessa la milza, il duodeno e/o lo stomaco, con metastasi nel fegato, nel peritoneo e/o nei linfonodi. I segni d'esordio sono variabili e di solito aspecifici; comprendono il dolore addominale e/o al fianco, una massa addominale palpabile, emorragie del tratto gastrointestinale superiore, ittero o alterazioni degli enzimi epatici nel siero, vomito, anoressia e/o perdita di peso."},"nl":{"name":"Sereus cystadenocarcinoom van prancreas","synonyms":["Pancreatisch sereus cystadenocarcinoom"],"definition":"Een zeer zeldzame, maligne, epitheeltumor van de pancreas bestaande uit cystische structuren begrensd door glycogeenrijke heldere cellen, geassocieerd met locale invasie, vaak met betrokkenheid van milt, duodenum en/of maag, en metastatische uitzaaiing naar lever, buikvlies en/of lymfeklieren. Presenterende symptomen zijn variabel en meestal niet specifiek, en omvatten buik- en/of flankpijn, tastbare abdominale massa, bloeding in het bovenste gastro-intestinale stelsel, geelzucht of abnormale leverenzymen in het serum, braken, anorexie en/of gewichtsverlies."},"pt":{"name":"Cistoadenocarcinoma seroso do pâncreas"},"pl":{"name":"Gruczolakotorbielakorak surowiczy trzustki","synonyms":["Pancreatic serous cystadenocarcinoma"]},"cs":{"name":"Serózní cystadenokarcinom pankreatu","synonyms":["Pankreatický serózní cystadenokarcinom"]},"uk":{"name":"Серозна цистаденокарцинома підшлункової залози","synonyms":["Серозна цистаденокарцинома підшлункової залози"],"definition":"A very rare, malignant, epithelial tumor of the pancreas composed of cystic structures lined by glycogen-rich clear cells, associated with local invasiveness often involving the spleen, duodenum and/or stomach and metastatic spread to the liver, peritoneum and/or lymph nodes. Presenting symptoms are variable and usually non-specific and include abdominal and/or flank pain, palpable abdominal mass, upper gastrointestinal bleeding, jaundice or abnormal serum liver enzymes, vomiting, anorexia and/or weight loss."},"zh":{"name":"胰腺浆液性囊腺癌","synonyms":["胰腺浆液性囊腺癌"],"definition":"胰腺的一种非常罕见的恶性上皮肿瘤，由富含糖原的透明细胞排列的囊性结构组成，与局部浸润相关，通常累及脾脏，十二指肠和/或胃，转移扩散至肝脏，腹膜和/或淋巴结。表现的症状是可变的，通常是非特异性的，包括腹部和/或胁腹疼痛，明显的腹部肿块，上消化道出血，黄疸或血清肝酶异常，呕吐，厌食和/或体重减轻。"},"tr":{"name":"Pankreasın seröz kistadenokarsinomu","synonyms":["Pankreasın seröz 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":"1624575808"},{"source":"MONDO","code":"0003630","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1335315","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":0.002,"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":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"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]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.001,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}