{"orpha":213625,"name":"Leiomyosarcoma of the corpus uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Leiomyosarcoma of the corpus uteri is a rare, malignant, mesenchymal tumor of smooth muscle origin characterized, histologically, by spindle and/or pleomorphic cells, often forming disorganized fascicles, with tumor cell necrosis and, macroscopically, by a large, soft, usually intramural mass with irregular borders and necrotic and hemorrhagic areas, located in the uterus. Presenting signs and symptoms typically include dysfunctional vaginal bleeding, vaginal discharge, palpable pelvic mass and/or pelvic pain/pressure. Changes in bowel habits, frequent or painful urination and hematuria may also be associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213625/","orphanet_url":"https://www.orpha.net/en/disease/detail/213625","names":{"fr":{"name":"Léiomyosarcome du corps de l'utérus","definition":"Tumeur mésenchymateuse rare, maligne, prenant naissance dans les muscles lisses, caractérisée sur la plan histologique par des cellules fusiformes et/ou pléomorphes, formant souvent des fascicules désorganisés, et par une nécrose des cellules tumorales et, sur le plan macroscopique, par une tumeur importante, molle, généralement intramurale, aux bords irréguliers et des zones nécrotiques et hémorragiques, localisée dans l'utérus. Les signes et symptômes observés sont généralement des saignements vaginaux dysfonctionnels, des pertes vaginales, une masse pelvienne perceptible à la palpation et/ou des douleurs/pressions pelviennes. Des changements d'habitudes intestinales, des mictions fréquentes ou douloureuses et une hématurie peuvent également être associés."},"de":{"name":"Leiomyosarkom des Corpus uteri"},"es":{"name":"Leiomiosarcoma de cuerpo de útero","definition":"El leiomiosarcoma del cuerpo de útero es un tumor mesenquimal maligno y poco frecuente del músculo liso caracterizado histológicamente por células fusiformes y/o pleomórficas, que a menudo forman fascículos desorganizados, con necrosis de células tumorales y, macroscópicamente, por una masa generalmente intramural, grande y blanda, con bordes irregulares y áreas necróticas y hemorrágicas, localizada en el útero. Los signos y síntomas de presentación suelen incluir sangrado vaginal disfuncional, flujo vaginal, masa pélvica palpable y/o dolor / presión pélvicos. También se pueden asociar cambios en los hábitos intestinales, micción frecuente o dolorosa y hematuria."},"it":{"name":"Leiomiosarcoma del corpo dell'utero","definition":"Il leiomiosarcoma del corpo dell'utero è un tumore mesenchimale maligno raro, che origina dalla muscolatura liscia, caratterizzato, dal punto di vista istologico, da cellule fusiformi e/o pleomorfe, che spesso si raggruppano in fasci disorganizzati, con necrosi delle cellule tumorali. Dal punto di vista macroscopico, si manifesta come massa molle di grosse dimensioni, di solito intramurale, con margini irregolari ed aree necrotiche ed emorragiche, localizzata nell'utero. In genere, i segni clinici d'esordio comprendono le emorragie e le perdite vaginali, la presenza di una massa pelvica palpabile e/o il dolore pelvico o la sensazione di pressione. Possono associarsi modificazioni delle abitudini intestinali, minzione frequente o dolorosa ed ematuria."},"nl":{"name":"Leiomyosarcoom van corpus uteri","synonyms":["Leiomyosarcoom van baarmoederlichaam"],"definition":"Leiomyosarcoom van het baarmoederlichaam is een zeldzame, maligne, mesenchymale tumor die is ontstaan uit gladde spieren. Histologisch wordt de tumor gekenmerkt door spoelvormige en/of pleomorfe cellen die vaak wanordelijke fasciculi vormen alsook necrose van tumorcellen, en macroscopisch door een grote, zachte, doorgaans intramurale massa met onregelmatige grenzen en necrotische en hemorragische gebieden die zich in de uterus bevindt. Zichtbare tekenen en symptomen omvatten doorgaans disfunctionele vaginale bloeding, vaginale afscheiding, tastbare pelviene massa en/of pijn/druk in het bekken. Veranderingen van de stoelgang, frequent of pijnlijk urineren, en hematurie kunnen ook geassocieerd zijn."},"pt":{"name":"Leiomiossarcoma do corpo uterino"},"pl":{"name":"Mięsak gładkokomórkowy trzonu macicy"},"cs":{"name":"Leiomyosarkom těla děložního"},"uk":{"name":"Лейоміосаркома тіла матки","definition":"Leiomyosarcoma of the corpus uteri is a rare, malignant, mesenchymal tumor of smooth muscle origin characterized, histologically, by spindle and/or pleomorphic cells, often forming disorganized fascicles, with tumor cell necrosis and, macroscopically, by a large, soft, usually intramural mass with irregular borders and necrotic and hemorrhagic areas, located in the uterus. Presenting signs and symptoms typically include dysfunctional vaginal bleeding, vaginal discharge, palpable pelvic mass and/or pelvic pain/pressure. Changes in bowel habits, frequent or painful urination and hematuria may also be associated."},"zh":{"name":"子宫体平滑肌肉瘤","definition":"子宫体平滑肌肉瘤是一种罕见的、恶性、间叶性、平滑肌起源的肿瘤，其组织学特征为梭形和/或多形性细胞，常形成杂乱的肌束伴肿瘤细胞坏死；肉眼上表现为子宫内大而软的壁内肿块，伴不规则边界和坏死出血区。本病的症状和体征通常包括功能失调性阴道出血、阴道分泌物、可触及的盆腔肿块和/或盆腔疼痛/压迫。曾报道与排便习惯改变，尿频或尿痛，血尿相关。"},"tr":{"name":"Korpus uteri leiomyosarkomu"}},"xrefs":[{"source":"ICD-10","code":"C54.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B58.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1872567422"},{"source":"MONDO","code":"0016262","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0280631","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10046799","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":213620,"name":"Sarcoma of the corpus uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}