{"orpha":104076,"name":"Leiomyosarcoma of small intestine","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Small bowel leiomyosarcoma is a rare type of small bowel malignancy, originating in the smooth muscle cells within the muscularis propria or the muscularis mucosa, most often found in the jejunum, and presenting with gastrointestinal bleeding and anemia and sometimes with other non-specific symptoms such as vomiting, nausea, abdominal pain and weakness and spreading to regional lymph nodes in 14% of cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-104076/","orphanet_url":"https://www.orpha.net/en/disease/detail/104076","names":{"fr":{"name":"Léiomyosarcome de l'instestin grêle","definition":"Le léiomyosarcome de l'intestin grêle est un type rare de tumeur maligne de l'intestin grêle. Il est issu des cellules musculaires lisses au sein de la propria musculaire ou de la muqueuse musculaire, et est retrouvé le plus souvent dans le jéjunum. Il se manifeste par une hémorragie gastro-intestinale et une anémie, et parfois par d'autres signes non spécifiques tels que des vomissements, des nausées, une douleur abdominale et une faiblesse, et s'étend aux ganglions lymphatiques régionaux dans 14% des cas."},"de":{"name":"Leiomyosarkom des Dünndarms","definition":"Das Dünndarm-Leiomyosarkom ist ein seltenes Malignom des Dünndarms. Es entsteht in glatten Muskelzellen der Muskularis oder Mukosa, am häufigsten im Jejunum. Symptome sind: Gastrointestinale Blutungen und Anämie, manchmal in Verbindung mit unspezifischen Symptomen wie Erbrechen, Übelkeit, Leibschmerzen. In 14% der Fälle sind auch regionale Lymphknoten befallen."},"es":{"name":"Leiomiosarcoma de intestino delgado","definition":"El leiomiosarcoma de intestino delgado es un tipo poco frecuente de cáncer de intestino delgado, que se origina en las células musculares lisas dentro de la propia musculatura o la mucosa muscular, hallado con más frecuencia en el yeyuno, y que se presenta con sangrado gastrointestinal y anemia y, algunas veces, con otros síntomas no específicos como vómitos, nausea, dolor abdominal y debilidad y que se propaga a los ganglios linfáticos regionales en el 14% de los casos."},"it":{"name":"Leiomiosarcoma dell'intestino tenue","definition":"Il leiomiosarcoma dell'intestino tenue è una forma rara di tumore intestinale, che origina nelle cellule dei muscoli lisci, all'interno della muscolare propria o della muscolaris mucosae, e si localizza prevalentemente nel digiuno. Si manifesta attraverso il sanguinamento gastrointestinale e l'anemia e, a volte, si accompagna a sintomi aspecifici, come il vomito, la nausea, il dolore addominale e la debolezza. Nel 14% dei casi si diffonde ai linfonodi regionali."},"nl":{"name":"Leiomyosarcoom van dunne darm","definition":"Dunne darm-leiomyosarcoom is een zeldzame aandoening aan de dunne darm die ontstaat in de gladde spiercellen van de muscularis propria of de muscularis mucosa, het vaakst wordt aangetroffen in het jejunum, en zich uit met gastro-intestinale bloedingen en anemie, en soms met andere niet-specifieke symptomen zoals braken, misselijkheid, pijn in de buik en slapte. In% van de gevallen is er verspreiding naar regionale lymfeklieren."},"pt":{"name":"Leiomiossarcoma do intestino curto"},"pl":{"name":"Mięsak gładkokomórkowy jelita cienkiego","definition":"Mięsak gładkokomórkowy jelita cienkiego jest rzadkim typem nowotworu złośliwego jelita cienkiego, pochodzącym z komórek mięśni gładkich w obrębie mięśniówki właściwej lub błony śluzowej mięśniowej, najczęściej zlokalizowanym w jelicie czczym i objawiającym się krwawieniem z przewodu pokarmowego i anemią, a czasami innymi niespecyficznymi objawy, takimi jak wymioty, nudności, ból brzucha czy osłabienie i rozprzestrzeniającym się w 14% przypadków do regionalnych węzłów chłonnych."},"cs":{"name":"Leiomyosarkom tenkého střeva"},"uk":{"name":"Лейоміосаркома тонкої кишки","definition":"Small bowel leiomyosarcoma is a rare type of small bowel malignancy, originating in the smooth muscle cells within the muscularis propria or the muscularis mucosa, most often found in the jejunum, and presenting with gastrointestinal bleeding and anemia and sometimes with other non-specific symptoms such as vomiting, nausea, abdominal pain and weakness and spreading to regional lymph nodes in 14% of cases."},"zh":{"name":"小肠平滑肌肉瘤","definition":"小肠平滑肌肉瘤是一种罕见的小肠恶性肿瘤,起源于固有肌层内的平滑肌细胞或肌黏膜，最常发现在空肠,伴消化道出血和贫血,14%的病例中有时伴其他非特异性症状,如呕吐、恶心、腹痛和虚弱、扩散到区域淋巴结。"},"tr":{"name":"İnce bağırsak leiomyosarkomu"}},"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":"2B58.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"389419378"},{"source":"MONDO","code":"0003360","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0920305","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19852","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10041127","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":423798,"name":"Mesenchymal tumor of small intestine","classifications":[152,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}