{"orpha":300557,"name":"Carcinoma of the ampulla of Vater","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Ampulloma","Ampullary carcinoma"],"definition":"Carcinoma of the ampulla of Vater is a rare malignant tumor originating from the ampulla of Vater that can present with symptoms of general fatigue, loss of appetite, weight loss, nausea, vomiting, abdominal pain and, most commonly, painless obstructive jaundice. The tumor is believed to arise from duodenal, biliary or pancreatic epilthelium, resulting in the respective histological types. In general, carcinoma of the ampulla of Vater has a better prognosis (5-year survival rate of 45%) than cancers of the distal bile duct and pancreas.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-300557/","orphanet_url":"https://www.orpha.net/en/disease/detail/300557","names":{"fr":{"name":"Carcinome de l'ampoule de Vater","synonyms":["Ampullome","Ampullome vatérien"],"definition":"Le carcinome de l'ampoule de Vater est une tumeur maligne rare issue de l'ampoule de Vater, et qui se présente par une fatigue générale, une perte d'appétit, une perte de poids, des nausées, des vomissements, une douleur abdominale et, plus fréquemment, un ictère obstructif indolore. La tumeur pourrait être issue de l'épithélium duodénal, biliaire ou pancréatique, ce qui conduit aux types histologiques respectifs. En général, le carcinome de l'ampoule de Vater a un meilleur pronostic que les cancers des voies biliaires distales et du pancréas (taux de survie à 5 ans de 45%)."},"de":{"name":"Karzinom der Ampulla Vateri","synonyms":["Ampullom"]},"es":{"name":"Carcinoma de la ampolla de Vater","synonyms":["Ampuloma","Carcinoma ampular"],"definition":"Es un tumor maligno poco frecuente que se origina en la ampolla de Vater y que puede presentar síntomas de fatiga general, pérdida de apetito y de peso, náuseas, vómitos, dolor abdominal y, más comúnmente, ictericia obstructiva indolora. Se cree que el tumor se origina en el epitelio duodenal, biliar o pancreático, lo que da como resultado los tipos histológicos respectivos. En general, el carcinoma de la ampolla de Vater tiene un mejor pronóstico (tasa de supervivencia a 5 años del 45%) que los cánceres del conducto biliar distal y del páncreas."},"it":{"name":"Carcinoma dell'ampolla di Vater","synonyms":["Ampulloma","Carcinoma ampollare"],"definition":"Il carcinoma dell'ampolla di Vater è un tumore maligno raro che si sviluppa dall'ampolla di Vater e può esordire con segni generici come l'affaticamento, la perdita di appetito, la perdita di peso, la nausea, il vomito, il dolore addominale e, soprattutto, l'ittero ostruttivo non associato a dolore. Si ritiene che il tumore abbia origine dall'epitelio duodenale, biliare o pancreatico, a cui corrispondono le diverse classificazioni istologiche. In generale, il carcinoma dell'ampolla di Vater ha una prognosi maggiormente favorevole (con un tasso sopravvivenza a 5 anni del 45%) rispetto ai tumori del dotto biliare distale e del pancreas."},"nl":{"name":"Carcinoom van ampul van Vater","synonyms":["Ampullair carcinoom","Ampulloom"],"definition":"Carcinoom van de ampul van Vater is een zeldzame maligne tumor die ontstaat uit de ampul van Vater en die zich kan voordoen met symptomen als algemene vermoeidheid, verlies van eetlust, gewichtsverlies, misselijkheid, braken, buikpijn, en, als meest voorkomende, pijnloze obstructieve geelzucht. De tumor komt vermoedelijk voort uit duodenaal, biliair of pancreatisch epitheel, met als resultaat de respectievelijke histologische types. Algemeen genomen heeft carcinoom van de ampul van Vater een betere prognose (5-jaarsoverleving van 45%) dan kankers van het distale galkanaal en de pancreas."},"pt":{"name":"Carcinoma da ampola de Vater"},"pl":{"name":"Rak brodawki Vatera","synonyms":["Ampulloma (rak brodawki Vatera)","Rak typu ampulloma (rak brodawki Vatera)"]},"cs":{"name":"Karcinom Vaterovy papily","synonyms":["Ampulární karcinom","Ampulom"]},"uk":{"name":"Карцинома ампули Ватера","synonyms":["Ампульна карцинома","Ампулома"],"definition":"Carcinoma of the ampulla of Vater is a rare malignant tumor originating from the ampulla of Vater that can present with symptoms of general fatigue, loss of appetite, weight loss, nausea, vomiting, abdominal pain and, most commonly, painless obstructive jaundice. The tumor is believed to arise from duodenal, biliary or pancreatic epilthelium, resulting in the respective histological types. In general, carcinoma of the ampulla of Vater has a better prognosis (5-year survival rate of 45%) than cancers of the distal bile duct and pancreas."},"zh":{"name":"Vater壶腹癌","synonyms":["壶腹癌"],"definition":"Vater壶腹癌是一种起源于壶腹的罕见恶性肿瘤，可表现为全身乏力、食欲不振、体重减轻、恶心、呕吐、腹痛，最常见的是无痛性梗阻性黄疸。肿瘤起源于十二指肠、胆道或胰腺上皮，可导致不同组织学类型。总的来说，壶腹癌预后（5年生存率为45%）优于远端胆管癌和胰腺癌。"},"tr":{"name":"Vater ampulla karsinomu","synonyms":["Ampulloma","Ampuller karsinom"]}},"xrefs":[{"source":"ICD-10","code":"C24.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C16","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"267067189"},{"source":"MONDO","code":"0017590","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0262401","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10048853","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":98059,"name":"Rare digestive tumor","classifications":[202]},{"orpha":97935,"name":"Rare gastroenterologic disease","classifications":[152]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.59,"area":"France","status":"Validated","source":"10656214[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.57,"area":"United Kingdom","status":"Validated","source":"3664230[PMID]"}],"phenotypes":null}