{"orpha":424046,"name":"Acinar cell carcinoma of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Pancreatic acinar cell carcinoma"],"definition":"A very rare, malignant, epithelial tumor of the pancreas characterized, macroscopically, by a usually large, well-circumscribed, fully or partially encapsulated, solid mass, often with hemorrhage, necrosis and cystic changes, in any portion of the pancreas and, histologically, by neoplastic cells with variable degrees of differentiation and morphology, ranging from acinar structures similar to normal pancreatic acini to large sheets of poorly differentiated neoplastic cells. Presenting symptoms are typically non-specific and include abdominal pain, weight loss, vomiting, nausea, and/or, less commonly, jaundice. Immunohistochemical evidence of acinar-specific products is observed. Association with Lynch syndrome, familial adenomatous polyposis, and pancreatic panniculitis has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424046/","orphanet_url":"https://www.orpha.net/en/disease/detail/424046","names":{"fr":{"name":"Carcinome à cellules acineuses du pancréas","synonyms":["Carcinome acinaire du pancréas","Carcinome acinaire pancréatique","Carcinome pancréatique à cellules acineuses"],"definition":"Tumeur épithéliale rare et maligne du pancréas caractérisée, sur le plan macroscopique, par une masse solide généralement large, bien circonscrite, entièrement ou partiellement encapsulée, souvent accompagnée d'une hémorragie, d'une nécrose et de lésions kystiques, siégeant à tout endroit du pancréas et, sur le plan histologique, par des cellules néoplasiques de degré de différentiation et de morphologie variables, allant de structures acineuses similaires à des acini pancréatiques normaux à de vastes nappes de cellules néoplasiques peu différentiées. Les symptômes initiaux ne sont généralement pas spécifiques, à type de douleurs abdominales, perte de poids, vomissements, nausées et/ou, moins fréquemment, d'ictère. L'examen immunohistochimique met en évidence des produits spécifiques aux cellules acineuses. Une association avec le syndrome de Lynch, la polypose adénomateuse familiale et la panniculite pancréatique a été rapportée."},"de":{"name":"Azinuszellkarzinom des Pankreas","synonyms":["Pankreaskarzinom, azinäres"]},"es":{"name":"Carcinoma de células acinares de páncreas","synonyms":["Carcinoma pancreático de células acinares"],"definition":"Es un tumor pancreático epitelial maligno muy poco frecuente caracterizado macroscópicamente por una masa sólida, por lo general, grande, bien delimitada, total o parcialmente encapsulada, que a menudo presenta hemorragia, necrosis y cambios quísticos, localizada en cualquier parte del páncreas. Histológicamente se caracteriza por células neoplásicas con grados variables de diferenciación y morfología, que van desde estructuras acinares similares a los acinos pancreáticos normales hasta grandes láminas de células neoplásicas poco diferenciadas. Por lo habitual, los síntomas iniciales son inespecíficos e incluyen dolor abdominal, pérdida de peso, vómitos, náuseas y/o, con menor frecuencia, ictericia. Se observan evidencias inmunohistoquímicas de productos acinares específicos. Se ha descrito asociación con el síndrome de Lynch, la poliposis adenomatosa familiar y la paniculitis pancreática."},"it":{"name":"Carcinoma a cellule acinose del pancreas","synonyms":["Carcinoma pancreatico a cellule acinose"],"definition":"È una neoplasia epiteliale maligna molto rara del pancreas caratterizzata, dal punto di vista macroscopico, da una massa solida, in genere di ampie dimensioni, ben circoscritta, completamente o parzialmente incapsulata, spesso associata ad emorragia, necrosi e alterazioni cistiche, localizzata in una qualsiasi porzione del pancreas. Dal punto di vista istologico, è caratterizzata da cellule neoplastiche che presentano diversi gradi di differenziazione e morfologia variabile, che comprendono le strutture acinari con caratteristiche simili ai normali acini pancreatici, fino ad ampi strati di cellule neoplastiche scarsamente differenziate. Di solito i sintomi d'esordio sono aspecifici e comprendono il dolore addominale, la perdita di peso, il vomito, la nausea e/o, più raramente, l'ittero. In alcuni pazienti si associa alla sindrome di Lynch, alla poliposi adenomatosa familiare e alla panniculite pancreatica."},"nl":{"name":"Acinuscelcarcinoom van pancreas","synonyms":["Acinuscelcarcinoom van alvleesklier","Pancreatisch acinuscelcarcinoom"],"definition":"Een zeer zeldzame, maligne epitheeltumor van de pancreas die macroscopisch gekarakteriseerd wordt door een meestal grote, goed begrensde, volledig of gedeeltelijk ingekapselde, solide massa, vaak met hemorragie, necrose en cystische veranderingen, in om het even welk deel van de pancreas, en histologisch door neoplastische cellen met variabele gradaties van differentiatie en morfologie, gaande van acinaire structuren gelijkaardig aan normale acini van de pancreas tot grote lagen met weinig gedifferentieerde neoplastische cellen. Presenterende symptomen zijn doorgaans niet specifiek en omvatten onder meer buikpijn, gewichtsverlies, braken, misselijkheid, en/of, minder gangbaar, geelzucht. Immunohistochemisch bewijs van acinus-specifieke producten wordt waargenomen. Associatie met syndroom van Lynch, familiale adenomateuze polypose, en pancreatische panniculitis werd reeds gerapporteerd."},"pt":{"name":"Carcinoma de células acinares do pâncreas"},"pl":{"name":"Rak z komórek groniastych trzustki","synonyms":["Pancreatic acinar cell carcinoma"]},"cs":{"name":"Karcinom pankreatu z acinárních buněk","synonyms":["Pankreatický karcinom z acinárních buněk"]},"uk":{"name":"Ацинарно-клітинний рак підшлункової залози","synonyms":["Ацинарно-клітинна карцинома підшлункової залози"],"definition":"A very rare, malignant, epithelial tumor of the pancreas characterized, macroscopically, by a usually large, well-circumscribed, fully or partially encapsulated, solid mass, often with hemorrhage, necrosis and cystic changes, in any portion of the pancreas and, histologically, by neoplastic cells with variable degrees of differentiation and morphology, ranging from acinar structures similar to normal pancreatic acini to large sheets of poorly differentiated neoplastic cells. Presenting symptoms are typically non-specific and include abdominal pain, weight loss, vomiting, nausea, and/or, less commonly, jaundice. Immunohistochemical evidence of acinar-specific products is observed. Association with Lynch syndrome, familial adenomatous polyposis, and pancreatic panniculitis has been reported."},"zh":{"name":"胰腺腺泡细胞癌","synonyms":["胰腺腺泡细胞癌"],"definition":"胰腺的一种非常罕见的恶性上皮肿瘤，大体上通常具有大的、界限清楚的、完全或部分包封的实性肿块，在胰腺的任何部分均可出现出血，坏死和囊性变化。在组织学上，肿瘤由分化程度和形态各异的肿瘤细胞组成，从类似于正常胰腺腺泡的腺泡结构到大片的分化差的肿瘤细胞。患者呈现的症状通常是非特异性的，包括腹痛，体重减轻，呕吐，恶心和/或（较不常见）黄疸。免疫组织化学检测可观察到腺泡特异性产物。据报道，该病与林奇综合征，家族性腺瘤性息肉病和胰腺脂膜炎相关。"},"tr":{"name":"Asiner hücreli pankreas karsinomu","synonyms":["Pankreatik asiner 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":"2C10.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"449785448"},{"source":"MONDO","code":"0006346","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0279661","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10073363","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":0.029,"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":0.016,"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":0.039,"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":0.017,"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":0.049,"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":0.039,"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":0.034,"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":0.006,"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":0.044,"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":0.083,"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":0.024,"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":0.01,"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":0.058,"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":0.028,"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":0.039,"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":0.07,"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":0.027,"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":0.005,"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":0.02,"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":0.05,"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":0.024,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}