{"orpha":213807,"name":"Leiomyosarcoma of the cervix uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Cervical leiomyosarcoma"],"definition":"Leiomyosarcoma of the cervix uteri is a rare, malignant mesenchymal tumor of smooth muscle origin, macroscopically appearing as a large, poorly circumscribed mass, often protruding from the cervical canal or expanding it circumferentially. The most common presenting symptoms are vaginal discharge or bleeding, pain in the lower abdomen and a bulky cervical mass. There is a reported tendency to metastatsize hematogenously, especially to the lungs, peritoneum, bones and the liver.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213807/","orphanet_url":"https://www.orpha.net/en/disease/detail/213807","names":{"fr":{"name":"Léiomyosarcome du col de l'utérus","synonyms":["Léiomyosarcome cervical"],"definition":"Le léiomyosarcome du col de l'utérus est une tumeur mésenchymateuse maligne et rare, issue du muscle lisse. Au niveau macroscopique, il se présente par une large masse peu circonscrite, qui protrude souvent à partir du canal cervical, ou s'y étend de manière circonférentielle. Les signes les plus fréquents sont une perte ou un saignement vaginal, une douleur dans le bas ventre et une masse cervicale volumineuse. Une tendance à la métastase par voie hématogène a été rapportée, surtout au niveau des poumons, du péritoine, des os et du foie."},"de":{"name":"Leiomyosarkom der Cervix uteri","synonyms":["Leiomyosarkom, zervikales"],"definition":"Das Leiomyosarkom des Gebärmutterhalses ist ein seltener, bösartiger mesenchymaler Tumor glatten Muskelursprungs, der makroskopisch als große, schlecht umschriebene Masse erscheint, oft aus dem Gebärmutterhalskanal herausragt oder ihn umlaufend erweitert. Die häufigsten Symptome sind Vaginalausfluss oder Blutungen, Schmerzen im Unterleib und eine voluminöse zervikale Masse. Es wird eine Tendenz zur hämatogenen Metastasierung gesehen, insbesondere in der Lunge, im Bauchfell, in den Knochen und in der Leber."},"es":{"name":"Leiomiosarcoma de cuello de útero","synonyms":["Leiomiosarcoma cervical"],"definition":"El leiomiosarcoma de cuello de útero es un tumor mesenquimal maligno, poco frecuente, de origen en el músculo liso, que se presenta macroscópicamente como una masa grande y pobremente circunscrita que a menudo sobresale desde el canal cervical o se expande de forma circunferencial. Los síntomas de presentación más comunes son flujo o sangrado vaginal, dolor en la parte inferior del abdomen y una masa cervical voluminosa. Se ha descrito una tendencia a metastatizar por vía hematógena, especialmente a pulmones, peritoneo, huesos e hígado."},"it":{"name":"Leiomiosarcoma della cervice uterina","synonyms":["Leiomiosarcoma cervicale"],"definition":"Il leiomiosarcoma della cervice uterina è un tumore mesenchimale maligno raro che origina dalla muscolatura liscia; a livello macroscopico, si presenta come una massa ampia e scarsamente circoscritta, che spesso sporge dal canale cervicale oppure ne allarga la circonferenza. I sintomi d'esordio più frequenti sono le perdite vaginali o le emorragie, il dolore nella parte inferiore dell'addome e la presenza di una massa cervicale di dimensioni notevoli. È stata osservata una tendenza alle metastasi per via ematica, in particolare ai polmoni, al peritoneo, alle ossa e al fegato."},"nl":{"name":"Leiomyosarcoom van cervix uteri","synonyms":["Cervicaal leiomyosarcoom","Leiomyosarcoom van baarmoederhals"],"definition":"Leiomyosarcoom van de cervix uteri is een zeldzame, kwaadaardige mesenchymale tumor met een oorsprong in het gladde spierweefsel die macroscopisch voorkomt als een groot, weinig afgelijnd gezwel dat vaak uitsteekt uit het baarmoederhalskanaal of de omtrek ervan doet toenemen. De meest voorkomende symptomen zijn vaginale afscheiding of bloeding, pijn in de onderbuik en een groot cervicaal gezwel. Er werd een tendens tot hematogene metastasering gerapporteerd, voornamelijk naar de longen, het buikvlies, de botten en de lever."},"pt":{"name":"Leiomiossarcoma do colo do útero"},"pl":{"name":"Mięsak głoadkokomórkowy szyjki macicy","synonyms":["Mięsak głoadkokomórkowy szyjki"]},"cs":{"name":"Leiomyosarkom hrdla děložního","synonyms":["Cervikální leiomyosarkom"]},"uk":{"name":"Лейоміосаркома шийки матки","synonyms":["Лейоміосаркома шийки матки"],"definition":"Leiomyosarcoma of the cervix uteri is a rare, malignant mesenchymal tumor of smooth muscle origin, macroscopically appearing as a large, poorly circumscribed mass, often protruding from the cervical canal or expanding it circumferentially. The most common presenting symptoms are vaginal discharge or bleeding, pain in the lower abdomen and a bulky cervical mass. There is a reported tendency to metastatsize hematogenously, especially to the lungs, peritoneum, bones and the liver."},"zh":{"name":"子宫颈平滑肌肉瘤","synonyms":["宫颈平滑肌肉瘤"],"definition":"子宫颈平滑肌肉瘤是一种罕见的、来源于平滑肌的恶性间叶性肿瘤，肉眼上表现为一个大的、边界不清的肿块，常从宫颈管突出或向周围扩张。最常见的症状是阴道分泌物或出血、下腹疼痛和宫颈部肿块。据报道有血液转移的倾向，尤其是转移至肺、腹膜、骨骼和肝脏。"},"tr":{"name":"Serviks uteri leiomyosarkomu","synonyms":["Servikal leiomyosarkom"]}},"xrefs":[{"source":"ICD-10","code":"C53.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C53.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C53.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B58.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1221958476"},{"source":"MONDO","code":"0016283","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4289817","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":213797,"name":"Sarcoma of cervix uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_23519508[PMID]"}],"phenotypes":null}