{"orpha":424039,"name":"Squamous cell carcinoma of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Pancreatic squamous cell carcinoma"],"definition":"A rare epithelial tumor of the exocrine pancreas, histologically characterized by presence of keratinization and/or intracellular bridges and lymphovascular and perineural invasion, as well as high metastatic potential. Patients present with upper abdominal and back pain, anorexia, weight loss, nausea, vomiting and jaundice.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424039/","orphanet_url":"https://www.orpha.net/en/disease/detail/424039","names":{"fr":{"name":"Carcinome épidermoïde du pancréas","synonyms":["Carcinome épidermoïde pancréatique"],"definition":"Une tumeur épithéliale rare du pancréas exocrine, caractérisé sur le plan histologique par la présence d'une kératinisation et/ou des ponts intracellulaires, par une invasion lymphovasculaire et périneurale, ainsi que par un fort potentiel métastatique. Les patients présentent une douleur lombaire et dans le haut du ventre, une anorexie, une perte de poids, des nausées, des vomissements et un ictère."},"de":{"name":"Plattenepithelkarzinom des Pankreas","synonyms":["Squamöses Karzinom des Pankreas"],"definition":"Ein seltener epithelialer Tumor der exokrinen Bauchspeicheldrüse, der histologisch durch Keratinisierung und/oder intrazelluläre Brücken und lymphovaskuläre und perineurale Invasion sowie ein hohes Metastasierungspotenzial gekennzeichnet ist. Die Patienten klagen über Schmerzen im Oberbauch und im Rücken, Appetitlosigkeit, Gewichtsverlust, Übelkeit, Erbrechen und Gelbsucht."},"es":{"name":"Carcinoma de células escamosas de páncreas","synonyms":["Carcinoma pancreático de células escamosas"],"definition":"Es un tumor epitelial poco frecuente del páncreas exocrino caracterizado histológicamente por la presencia de queratinización y/o puentes intracelulares con invasión linfovascular y perineural, así como por un elevado potencial metastásico. Los afectados presentan dolor en la parte superior del abdomen y dolor de espalda, anorexia, pérdida de peso, náuseas, vómitos e ictericia."},"it":{"name":"Carcinoma a cellule squamose del pancreas","synonyms":["Carcinoma pancreatico a cellule squamose"],"definition":"Il carcinoma a cellule squamose del pancreas è una neoplasia epiteliale rara del pancreas esocrino. Dal punto di vista istologico, è caratterizzato dalla presenza di cheratinizzazione e/o ponti intracellulari e dalla invasione linfovascolare e perineurale; il potenziale metastatico è elevato. I pazienti presentano dolore nella parte superiore dell'addome e alla schiena, anoressia, perdita di peso, nausea, vomito e ittero."},"nl":{"name":"Plaveiselcelcarcinoom van pancreas","synonyms":["Pancreatisch plaveiselcelcarcinoom","Plaveiselcelcarcinoom van alvleesklier"],"definition":"Een zeldzame epitheliale tumor van exocriene pancreas, histologisch gekenmerkt door de aanwezigheid van keratinisatie en/of intracellulaire bruggen en lymfovasculaire en perineurale invasie, alsook een hoog metastatisch potentieel. Patiënten vertonen pijn aan bovenste deel van abdomen en aan rug, anorexie, gewichtsverlies, misselijkheid, braken en geelzucht."},"pt":{"name":"Carcinoma de células escamosas do pâncreas"},"pl":{"name":"Rak kolczystokomórkowy trzustki","synonyms":["Pancreatic squamous cell carcinoma"],"definition":"*Rak kolczystokomórkowy trzustki jest rzadkim guzem nabłonkowym wywodzącym się z komórek zewnątrzwydzielniczych trzustki. Histologicznie charakteryzuje się obecnością keratynizacji i/lub wewnątrzkomórkowych mostków oraz nacieków w naczyniach limfatycznych i okolicach okołonerwowych, a także dużym potencjałem do tworzenia przerzutów. Objawy zgłaszane przez pacjentów to bóle pleców oraz w nadbrzuszu, jadłowstręt, utrata masy ciała, nudności, wymioty i żółtaczka."},"cs":{"name":"Dlaždicobuněčný karcinom pankreatu","synonyms":["Pankreatický dlaždicobuněčný karcinom"]},"uk":{"name":"Плоскоклітинний рак підшлункової залози","synonyms":["Плоскоклітинний рак підшлункової залози"],"definition":"A rare epithelial tumor of the exocrine pancreas, histologically characterized by presence of keratinization and/or intracellular bridges and lymphovascular and perineural invasion, as well as high metastatic potential. Patients present with upper abdominal and back pain, anorexia, weight loss, nausea, vomiting and jaundice."},"zh":{"name":"胰腺鳞状细胞癌","synonyms":["胰腺鳞状细胞癌"],"definition":"胰腺鳞状细胞癌是罕见的外分泌胰腺上皮肿瘤，其组织学特征是存在角质化和/或细胞内桥，淋巴管和神经周浸润，以及高转移潜力。患者可出现上腹部和背部疼痛，厌食，体重减轻，恶心，呕吐和黄疸。"},"tr":{"name":"Pankreasın skuamöz hücreli karsinomu","synonyms":["Pankreatik skuamöz hücreli karsinom"]}},"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.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018521","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2675993","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":0.023,"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":0.034,"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":0.019,"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":0.06,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.058,"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":0.003,"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":0.008,"area":"Poland","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":"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":0.005,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.027,"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":0.011,"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":0.023,"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":0.044,"area":"Austria","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":0.027,"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":0.028,"area":"Croatia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.045,"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":0.028,"area":"Estonia","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":0.015,"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":0.032,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}