{"orpha":104075,"name":"Adenocarcinoma of the small intestine","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Adenocarcinoma of the small bowel"],"definition":"Small bowel adenocarcinoma (SBA) is a rare small intestinal malignancy, most commonly located in the duodenum (55% of cases) but also rarely in the jejunum and ileum, which is usually discovered at an advanced stage in the 6th to 7th decade of life due to non-specific symptoms at presentation such as nausea, abdominal pain and weight loss. In some cases it is asymptomatic, and therefore usually has a poor prognosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-104075/","orphanet_url":"https://www.orpha.net/en/disease/detail/104075","names":{"fr":{"name":"Adénocarcinome de l'intestin grêle","definition":"L'adénocarcinome de l'intestin grêle est une tumeur maligne rare de l'intestin grêle, localisée le plus souvent dans le duodénum (55% des cas), et plus rarement dans le jéjunum et l'iléum. Il est généralement découvert à un âge avancé (pendant la sixième ou la septième décennie) devant des signes non spécifiques, tels qu'une nausée, des douleurs abdominales et une perte de poids. Il est asymptomatique dans certains cas, et a de ce fait un mauvais pronostic."},"de":{"name":"Adenokarzinom des Dünndarms","definition":"Das Dünndarm-Adenokarzinom (SBA) ist ein seltenes intestinales Malignom, liegt meist (55% der Fälle) im Duodenum, seltener auch im Jejunum und Ileum. Es wird in der Regel im fortgeschrittenen Alter (6.-7. Lebensjahrzehnt) beim Auftreten unspezifischer Symptome (Übelkeit, Leibschmerzen, Gewichtsverlust) entdeckt. Einige Fälle bleiben symptomlos und haben dann meist eine schlechte Prognose."},"es":{"name":"Adenocarcinoma de intestino delgado","definition":"El adenocarcinoma de intestino delgado (SBA) es un cáncer de intestino delgado poco frecuente, localizado con más frecuencia en el duodeno (55% de los casos) pero también con menos frecuencia en el yeyuno y el íleon, que suele descubrirse en una etapa avanzada en la 6ª o 7ª década de vida por la presencia de síntomas no específicos como nausea, dolor abdominal y pérdida de peso. En algunos casos es asintomático, y por lo tanto suele tener un mal pronóstico."},"it":{"name":"Adenocarcinoma dell'intestino tenue","definition":"L'adenocarcinoma dell'intestino tenue (SBA) è un tumore maligno raro dell'intestino tenue, localizzato per lo più nel duodeno (55% dei casi), ma anche nel digiuno e nell'ileo. Viene di solito diagnosticato in uno stadio avanzato, nella sesta-settima decade di vita, stante la presenza di sintomi non specifici, come la nausea, il dolore addominale e la perdita di peso. In alcuni casi, è asintomatico e, di conseguenza, ha di solito una prognosi sfavorevole."},"nl":{"name":"Adenocarcinoom van dunne darm","definition":"Dunne darm-adenocarcinoom (SBA) is een zeldzame aandoening aan de dunne darm, meestal gesitueerd in het duodenum (55% van de gevallen) maar soms ook in het jejunum en ileum, die meestal ontdekt wordt in een gevorderd stadium in het 6de of 7de levensdecennium als gevolg van niet-specifieke symptomen bij de manifestatie zoals misselijkheid, pijn in de buik en gewichtsverlies. In sommige gevallen is het asymptomatisch, en daardoor is de prognose meestal ongunstig."},"pt":{"name":"Adenocarcinoma do intestino curto"},"pl":{"name":"Gruczolakorak jelita cienkiego","synonyms":["Adenocarcinoma of the small bowel"]},"cs":{"name":"Adenokarcinom tenkého střeva","synonyms":["Adenocarcinoma of the small bowel"]},"uk":{"name":"Аденокарцинома тонкої кишки","synonyms":["Аденокарцинома тонкої кишки"],"definition":"Small bowel adenocarcinoma (SBA) is a rare small intestinal malignancy, most commonly located in the duodenum (55% of cases) but also rarely in the jejunum and ileum, which is usually discovered at an advanced stage in the 6th to 7th decade of life due to non-specific symptoms at presentation such as nausea, abdominal pain and weight loss. In some cases it is asymptomatic, and therefore usually has a poor prognosis."},"zh":{"name":"小肠腺癌","synonyms":["小肠腺瘤"],"definition":"小肠腺癌(SBA)是一种罕见的小肠恶性肿瘤,最常见的是位于十二指肠(55%的病例),但也少数在空肠和回肠,通常由于在60-70岁由于非特异性症状表现如恶心、腹痛和体重下降被发现。有些病例是无症状的，因此通常预后较差。"},"tr":{"name":"İnce bağırsak adenokarsinomu","synonyms":["İnce bağırsakta adenokarsinom"]}},"xrefs":[{"source":"ICD-10","code":"C17.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B80.20","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1369513329"},{"source":"MONDO","code":"0003198","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0278803","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19851","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10073373","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":423957,"name":"Rare carcinoma of small intestine","classifications":[152,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and 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incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.464,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.36,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.574,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.657,"area":"France","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.551,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.724,"area":"Iceland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.564,"area":"Ireland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.79,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.265,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.277,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.377,"area":"Malta","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.788,"area":"Norway","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.219,"area":"Poland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.743,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.462,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.4,"area":"Slovenia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.477,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.76,"area":"Switzerland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.626,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.679,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}