{"orpha":423968,"name":"Squamous cell carcinoma of the small intestine","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Squamous cell carcinoma of the small bowel"],"definition":"Squamous cell carcinoma of the small intestine is an extremely rare, malignant, epithelial tumor of the small intestine (most often localized in the duodenum). Presenting symptoms are often nonspecific, such as weight loss, epigastric pain, anorexia, weakness, fatigue, vomiting and abdominal distension, and vary depending on localization of the tumor. Gastrointestinal bleeding and perforation may occur in advanced cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-423968/","orphanet_url":"https://www.orpha.net/en/disease/detail/423968","names":{"fr":{"name":"Carcinome épidermoïde de l'intestin grêle","definition":"Une tumeur maligne épithéliale extrêmement rare de l'intestin grêle (située le plus souvent au niveau du duodénum). Les signes de présentation sont souvent non spécifiques, comme une perte de poids, une douleur épigastrique, une anorexie, une faiblesse, une fatigue, des vomissements et une distension abdominale. Ils varient selon la localisation de la tumeur. Une perforation gastro-intestinale et un saignement peuvent survenir dans les cas avancés."},"de":{"name":"Plattenepithelkarzinom des Dünndarms","synonyms":["Squamöses Karzinom des Dünndarms"],"definition":"Das Plattenepithelkarzinom des Dünndarms ist ein extrem seltener, bösartiger, epithelialer Tumor des Dünndarms (am häufigsten im Zwölffingerdarm lokalisiert). Die Symptome sind oft unspezifisch (z. B. Gewichtsverlust, epigastrische Schmerzen, Anorexie, Schwäche, Müdigkeit, Erbrechen und Blähbauch) und variieren je nach Lokalisation des Tumors. In fortgeschrittenen Fällen können gastrointestinale Blutungen und Perforationen auftreten."},"es":{"name":"Carcinoma de células escamosas de intestino delgado","definition":"El carcinoma de células escamosas de intestino delgado es un tumor epitelial maligno muy poco frecuente del intestino delgado (localizado con frecuencia en el duodeno). Los síntomas de presentación son a menudo inespecíficos, tales como pérdida de peso, dolor epigástrico, anorexia, debilidad, fatiga, vómitos y distensión abdominal, y varían según la localización del tumor. En casos avanzados puede aparecer hemorragia gastrointestinal y perforación."},"it":{"name":"Carcinoma a cellule squamose dell'intestino tenue","synonyms":["Carcinoma a cellule squamose del piccolo intestino"],"definition":"Il carcinoma a cellule squamose dell'intestino tenue è un tumore epiteliale maligno molto raro, localizzato per lo più nel duodeno. I sintomi d'esordio sono spesso aspecifici e dipendono dalla localizzazione del tumore; comprendono la perdita di peso, il dolore epigastrico, l'anoressia, la debolezza, l'affaticamento, il vomito e la distensione addominale. Negli stadi avanzati possono verificarsi emorragie e perforazioni del tratto gastrointestinale."},"nl":{"name":"Plaveiselcelcarcinoom van dunne darm","definition":"Plaveiselcelcarcinoom van de dunne darm is een uiterst zeldzame, maligne, epitheliale tumor van de dunne darm (meestal gelokaliseerd in het duodenum). Aanwezige symptomen zijn vaak niet specifiek, zoals gewichtsverlies, epigastrische pijn, anorexia, zwakte, vermoeidheid, braken en abdominale distensie, en variëren afhankelijk van de lokalisatie van de tumor. Gastro-intestinale bloeding en perforatie kunnen voorkomen in vergevorderde gevallen."},"pt":{"name":"Carcinoma de células escamosas do intestino delgado"},"pl":{"name":"Rak łuskowaty jelita cienkiego","synonyms":["Squamous cell carcinoma of the small bowel"]},"cs":{"name":"Dlaždicobuněčný karcinom tenkého střeva","synonyms":["Squamous cell carcinoma of the small bowel"]},"uk":{"name":"Плоскоклітинний рак тонкої кишки","synonyms":["Плоскоклітинний рак тонкого кишечника"],"definition":"Squamous cell carcinoma of the small intestine is an extremely rare, malignant, epithelial tumor of the small intestine (most often localized in the duodenum). Presenting symptoms are often nonspecific, such as weight loss, epigastric pain, anorexia, weakness, fatigue, vomiting and abdominal distension, and vary depending on localization of the tumor. Gastrointestinal bleeding and perforation may occur in advanced cases."},"zh":{"name":"小肠鳞状细胞癌","synonyms":["小肠鳞状细胞癌"],"definition":"小肠鳞状细胞癌是一种非常罕见，恶性的小肠上皮肿瘤（最常见于十二指肠）。表现的症状通常是非特异性的，例如体重减轻，上腹痛，厌食，肌无力，疲倦，呕吐和腹胀。症状取决于肿瘤的位置。晚期病例可能发生胃肠道出血和穿孔。"},"tr":{"name":"İnce bağırsakta skuamöz hücreli karsinom","synonyms":["İnce bağırsakta skuamöz hücreli karsinom"]}},"xrefs":[{"source":"ICD-10","code":"C17.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C17.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C17.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C17.3","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C17.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B80.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018509","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707899","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","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.008,"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":0.008,"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":0.008,"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":0.002,"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":0.013,"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":0.002,"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":0.008,"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":0.004,"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":0.012,"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":0.007,"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":0.022,"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":0.003,"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":0.003,"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":0.005,"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":0.011,"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":0.002,"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":0.012,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}