{"orpha":64720,"name":"Leiomyosarcoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare soft tissue sarcoma characterized by a malignant space-occupying lesion most commonly located in the retroperitoneum or the inferior vena cava, but also other soft tissues, and composed of cells showing distinct features of smooth muscle cells. The tumor presents with mass effect depending on the location. It is capable of both local recurrence and distant metastasis, while lymph node metastasis is rare. Prognosis largely depends on tumor location and size.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-64720/","orphanet_url":"https://www.orpha.net/en/disease/detail/64720","names":{"fr":{"name":"Léiomyosarcome","definition":"Sarcome rare des tissus mous caractérisé par une tumeur maligne occupant l'espace, située le plus souvent dans le rétropéritoine ou la veine cave inférieure, mais également dans d'autres tissus mous. Elle est composée de cellules présentant des caractéristiques distinctes des cellules musculaires lisses. La tumeur exerce un effet de masse tumorale en fonction de sa localisation. Elle peut récidiver localement et métastaser à distance, les métastases affectant rarement les ganglions lymphatiques. Le pronostic varie selon la localisation et la taille de la tumeur."},"de":{"name":"Leiomyosarkom","definition":"Ein seltenes Weichteilsarkom, bei dem es sich um eine bösartige, raumfordernde Läsion handelt, die meist im Retroperitoneum oder in der unteren Hohlvene, aber auch in anderen Weichteilen vorkommt und aus Zellen besteht, die deutliche Merkmale von glatten Muskelzellen aufweisen. Der Tumor zeigt je nach Lokalisation eine Gewebeverdichtung. Er kann sowohl lokal rezidivieren als auch Fernmetastasen bilden, während Lymphknotenmetastasen selten sind. Die Prognose hängt weitgehend von der Lage und Größe des Tumors ab."},"es":{"name":"Leiomiosarcoma","definition":"Es un sarcoma de tejidos blandos poco frecuente caracterizado por una lesión maligna ocupante de espacio, localizada con mayor frecuencia en el retroperitoneo o en la vena cava inferior, pudiendo aparecer también en otros tejidos blandos. Está compuesto por células que muestran características distintivas de las células del músculo liso. El tumor se presenta con un efecto masa dependiente de la localización. Es capaz tanto de desarrollar recidivas locales como de metastatizar a distancia, aunque la metástasis a los ganglios linfáticos es poco frecuente. El pronóstico depende, en gran medida, de la localización y del tamaño del tumor."},"it":{"name":"Leiomiosarcoma","definition":"Si tratta di un sarcoma raro dei tessuti molli, caratterizzato da una lesione maligna voluminosa, localizzata prevalentemente nel retroperitoneo o nella vena cava inferiore, ma anche in altri tessuti molli, composta da cellule che presentano le caratteristiche tipiche delle cellule della muscolatura liscia. A seconda della localizzazione, il tumore può esercitare un effetto massa. Può associarsi sia a recidive locali che a metastasi, ma quelle ai linfonodi sono rare. La prognosi dipende soprattutto dalla localizzazione e delle dimensioni del tumore."},"nl":{"name":"Leiomyosarcoom","definition":"Een zeldzaam wekedelensarcoom, gekarakteriseerd door een maligne, ruimte-innemende laesie die doorgaans is gelokaliseerd in retroperitoneum of vena cava inferior, maar mogelijk ook in andere weke delen, en bestaat uit cellen met typische kenmerken van gladde spiercellen. De tumor vertoont massa-effect, afhankelijk van de locatie. Zowel lokale recidive en verre metastase zijn mogelijk, terwijl uitzaaiing naar lymfeklieren zeldzaam is. De prognose hangt grotendeels af van locatie en omvang van de tumor."},"pt":{"name":"Leiomiosarcoma"},"pl":{"name":"Mięsak gładkokomórkowy"},"cs":{"name":"Leiomyosarkom"},"uk":{"name":"Лейоміосаркома","definition":"A rare soft tissue sarcoma characterized by a malignant space-occupying lesion most commonly located in the retroperitoneum or the inferior vena cava, but also other soft tissues, and composed of cells showing distinct features of smooth muscle cells. The tumor presents with mass effect depending on the location. It is capable of both local recurrence and distant metastasis, while lymph node metastasis is rare. Prognosis largely depends on tumor location and size."},"zh":{"name":"平滑肌肉瘤"},"tr":{"name":"Leiomyosarkom"}},"xrefs":[{"source":"ICD-10","code":"C49.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0005058","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D007890","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0023269","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6880","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10024189","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":289656,"name":"Epstein-Barr Virus-associated mesenchymal tumor","classifications":[202]},{"orpha":3394,"name":"Soft tissue sarcoma","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"WHO Classification of Tumours[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"WHO Classification of Tumours[OTHER]"}],"phenotypes":null}