{"orpha":424080,"name":"Undifferentiated carcinoma with osteoclast-like giant cells of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["OGCT of pancreas","Osteoclastic giant cell tumor of pancreas","Pancreatic osteoclastic giant cell tumor","Pancreatic undifferentiated carcinoma with osteoclast-like giant cells","Undifferentiated carcinoma of pancreas with osteoclast-like giant cells"],"definition":"A rare type of pancreatic ductal adenocarcinoma characterized by composition of non-neoplastic osteoclast-like multinucleated giant cells, a mononuclear histiocytic component, and the neoplastic cells, which vary from spindle-shaped to epithelioid and can be very large and pleomorphic. Clinical features of pancreatic ductal adenocarcinoma include abdominal pain, nausea, weight loss, jaundice, and new-onset diabetes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424080/","orphanet_url":"https://www.orpha.net/en/disease/detail/424080","names":{"fr":{"name":"Carcinome indifférencié à cellules géantes de type ostéoclastique du pancréas","synonyms":["Carcinome pancréatique indifférencié à cellules géantes de type ostéoclastique"],"definition":"Type rare d'adénocarcinome canalaire du pancréas caractérisé par la présence de cellules géantes multinucléées non tumorales de type ostéoclastique, d'une composante histiocytaire mononucléaire et de cellules néoplasiques, dont la forme varie de fusiforme à épithélioïde et qui peuvent être de grande taille et pléomorphes. Les signes cliniques de l'adénocarcinome canalaire du pancréas comprennent des douleurs abdominales, des nausées, une perte de poids, un ictère et un diabète d'apparition récente."},"de":{"name":"Undifferenziertes Karzinom des Pankreas mit osteoklastenähnlichen Riesenzellen","synonyms":["OGCT des Pankreas","Osteoklastischer Riesenzelltumor des Pankreas","Undifferenziertes Pankreaskarzinom mit osteoklastenähnlichen Riesenzellen"],"definition":"Eine seltene Form des duktalen Adenokarzinoms des Pankreas, die sich durch eine Zusammensetzung aus nicht-neoplastischen, osteoklastischen, vielkernigen Riesenzellen, einer mononukleären histiozytären Komponente und neoplastischen Zellen auszeichnet, die spindelförmig bis epitheloid, sehr groß und pleomorph sein können. Zu den klinischen Merkmalen des duktalen Adenokarzinoms des Pankreas gehören Bauchschmerzen, Übelkeit, Gewichtsverlust, Gelbsucht und neu auftretender Diabetes."},"es":{"name":"Carcinoma indiferenciado de páncreas con células gigantes tipo osteoclasto","synonyms":["Carcinoma pancreático indiferenciado con células gigantes tipo osteoclasto","TCGO de páncreas","Tumor de células gigantes osteoclásticas de páncreas","Tumor pancreático de células gigantes osteoclásticas"],"definition":"Es un tipo poco frecuente de adenocarcinoma ductal de pancreas caracterizado por una composición de células gigantes multinucleadas similares a osteoclastos no neoplásicos, un componente histiocítico mononuclear y células neoplásicas, que varían de fusiformes a epitelioides y que pueden ser muy grandes y pleomórficas. Las características clínicas del adenocarcinoma ductal de pancreas incluyen dolor abdominal, náuseas, pérdida de peso, ictericia y diabetes de inicio reciente."},"it":{"name":"Carcinoma non differenziato del pancreas con cellule giganti simili ad osteoclasti","synonyms":["Carcinoma pancreatico non differenziato con cellule giganti simili ad osteoclasti","OGCT del pancreas","Tumore osteoclastico a cellule giganti del pancreas","Tumore pancreatico osteoclastico a cellule giganti"]},"nl":{"name":"Ongedifferentieerd carcinoom met osteoclastachtige reuscellen van pancreas","synonyms":["OGCT van pancreas","Ongedifferentieerd carcinoom van pancreas met osteoclastachtige reuscellen","Osteoclastische reusceltumor van pancreas","Pancreatisch ongedifferentieerd carcinoom met osteoclastachtige reuscellen"],"definition":"Een zeldzaam type van adenocarcinoom van ductus pancreaticus, gekenmerkt door een samenstelling van niet-neoplastische osteoclast-achtige multinucleaire reuscellen, een mononucleaire histiocytische component, en neoplastische cellen die variëren van spoelvormig tot epithelioïd en die zeer groot en pleomorf kunnen zijn. Klinische kenmerken van adenocarcinoom van pancreasafvoergang zijn onder meer abdominale pijn, misselijkheid, gewichtsverlies, geelzucht, en nieuw optredende diabetes."},"pt":{"name":"Tumor osteoclástico de células gigantes do pâncreas","synonyms":["Carcinoma pancreático indiferenciado com células gigantes osteoclasto-like","OGCT pancreático"],"definition":"Tipo raro de adenocarcinoma ductal pancreático caracterizado pela composição de células gigantes multinucleadas não neoplásicas semelhantes a osteoclastos, um componente histiocítico mononuclear e células neoplásicas, que variam de fusiformes a epitelióides e podem ser muito grandes e pleomórficas. As características clínicas do adenocarcinoma ductal pancreático incluem dor abdominal, náuseas, perda de peso, icterícia e diabetes de início recente."},"pl":{"name":"Osteoklastyczny olbrzymiokomórkowy guz trzustki","synonyms":["Niezróżnicowany rak trzustki z podobnymi do osteoklastów komórkami olbrzymimi","OGCT trzustki","Rak niezróżnicowany trzustki zbudowany z komórek olbrzymich podobnych do osteoklastów"]},"cs":{"name":"Osteoklastický obrovskobuněčný tumor pankreatu","synonyms":["OGCT pankreatu","Pankreatický osteoklastický obrovskobuněčný tumor","Pankreatický nediferencovaný karcinom s osteoklastoidními obrovskými buňkami","Nediferencovaný karcinom pankreatu s osteoklastoidními obrovskými buňkami"]},"uk":{"name":"Недиференційована карцинома з остеокластоподібними гігантськими клітинами підшлункової залози","synonyms":["OGCT підшлункової залози","Остеокластична гігантоклітинна пухлина підшлункової залози","Остеокластична гігантоклітинна пухлина підшлункової залози","Недиференційована карцинома підшлункової залози з остеокластоподібними гігантськими клітинами","Недиференційована карцинома підшлункової залози з остеокластоподібними гігантськими клітинами"],"definition":"A rare type of pancreatic ductal adenocarcinoma characterized by composition of non-neoplastic osteoclast-like multinucleated giant cells, a mononuclear histiocytic component, and the neoplastic cells, which vary from spindle-shaped to epithelioid and can be very large and pleomorphic. Clinical features of pancreatic ductal adenocarcinoma include abdominal pain, nausea, weight loss, jaundice, and new-onset diabetes."},"zh":{"name":"胰腺破骨细胞样巨细胞瘤","synonyms":["未分化的胰腺破骨细胞样瘤"]},"tr":{"name":"Pankreasın osteoklastik dev hücreli tümörü","synonyms":["OGCT pankreası","Pankreatik osteoklastik dev hücreli tümör","Osteoklast ile seyreden dev hücreli pankreatik farklılaşmamış karsinom","Osteoklast ile seyreden dev hücreli farklılaşmamış pankreas karsinomu"]}},"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":"attributed","validated":true},{"source":"MONDO","code":"0006479","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2007059","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":0.006,"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":0.003,"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":0.001,"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":0.043,"area":"Iceland","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":"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":"Lithuania","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":"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":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}