{"orpha":52994,"name":"Orbital leiomyoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Orbital leiomyoma is a rare benign smooth muscle tumor arising from the walls of orbital vessels characterized by its slow growth and well encapsulated nature. It is usually located in an extraconal position, commonly manifesting with painless proptosis. The tumor is composed of spindle cells arranged in a fibrous stroma rich in dilated sinusoidal capillaries. The nuclei of tumor cells are oval with blunted ends and there are no mitotic figures. Orbital leiomyoma when excised has excellent prognosis for vision and life. One case of orbital leiomyosarcoma that possibly represents sarcomatous change in an orbital leiomyoma following radiation treatment has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-52994/","orphanet_url":"https://www.orpha.net/en/disease/detail/52994","names":{"fr":{"name":"Léiomyome orbitaire","definition":"Tumeur bénigne rare des muscles lisses prenant naissance dans les parois des vaisseaux orbitaires, caractérisée par une croissance lente et un aspect bien encapsulé. Située généralement en position extracônique, elle se manifeste souvent par une proptose indolore. La tumeur est composée de cellules fusiformes disposées dans un stroma fibreux riche en capillaires sinusoïdaux dilatés. Les noyaux des cellules tumorales sont ovales aux extrémités émoussées et ne présentent aucune figure mitotique. L'exérèse du léiomyome orbitaire est associée à un excellent pronostic concernant l'espérance de vie et la vision. Un cas de léiomyosarcome orbital qui pourrait représenter une transformation sarcomateuse d'un léiomyome orbital à la suite d'une radiothérapie a été rapporté."},"de":{"name":"Leiomyom, orbitales"},"es":{"name":"Leiomioma orbital","definition":"Es un tumor benigno poco frecuente del músculo liso que surge de las paredes de los vasos orbitarios y se caracteriza por su crecimiento lento y su naturaleza bien encapsulada. Suele localizarse en posición extraconal, manifestándose comúnmente con proptosis indolora. El tumor está compuesto por células fusiformes dispuestas en un estroma fibroso rico en capilares sinusoidales dilatados. Los núcleos de las células tumorales son ovalados con extremos romos y sin figuras mitóticas. El leiomioma orbitario, cuando se extirpa, tiene un pronóstico excelente para la visión y la esperanza de vida. Se ha registrado un caso de leiomiosarcoma orbitario que posiblemente represente un cambio sarcomatoso en un leiomioma orbitario después del tratamiento con radiación."},"it":{"name":"Leiomioma orbitale"},"nl":{"name":"Orbitaal leiomyoom","definition":"Een zeldzame goedaardige tumor van gladde spier, ontstaan uit wanden van orbitale bloedvaten, en gekenmerkt door trage groei en goede inkapseling. Het bevindt zich meestal in een extraconale positie, en manifesteert zich doorgaans met pijnloze proptosis. De tumor bestaat uit spoelcellen gerangschikt in een fibreus stroma rijk aan gedilateerde sinusoïdale capillairen. Kernen van tumorcellen zijn ovaal met stompe uiteinden, en er zijn geen mitotische figuren. Na excisie van orbitaal leiomyoom is de prognose voor zicht en leven excellent. Er werd één geval gerapporteerd van orbitaal leiomyosarcoom dat mogelijk een sarcomateuze verandering in een orbitaal leiomyoom na behandeling met straling vertegenwoordigt."},"pt":{"name":"Leiomioma da órbita"},"pl":{"name":"Mięsak gładkokomórkowy oczodołu"},"cs":{"name":"Leiomyom orbity"},"uk":{"name":"Орбітальна лейоміома","definition":"Orbital leiomyoma is a rare benign smooth muscle tumor arising from the walls of orbital vessels characterized by its slow growth and well encapsulated nature. It is usually located in an extraconal position, commonly manifesting with painless proptosis. The tumor is composed of spindle cells arranged in a fibrous stroma rich in dilated sinusoidal capillaries. The nuclei of tumor cells are oval with blunted ends and there are no mitotic figures. Orbital leiomyoma when excised has excellent prognosis for vision and life. One case of orbital leiomyosarcoma that possibly represents sarcomatous change in an orbital leiomyoma following radiation treatment has been reported."},"zh":{"name":"眼眶平滑肌瘤"},"tr":{"name":"Orbital leiomyom"}},"xrefs":[{"source":"ICD-10","code":"D31.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2F36.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"614302400"},{"source":"MONDO","code":"0018885","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4305000","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":101950,"name":"Rare eye tumor","classifications":[189,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":26.0,"area":"Worldwide","status":"Validated","source":"23562416[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_23562416[PMID]"}],"phenotypes":null}