{"orpha":289685,"name":"Myopericytoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare soft tissue tumor characterized by a benign subcutaneous lesion composed of oval-to-spindle shaped myoid appearing cells with a tendency for concentric perivascular growth. The tumor usually presents as a painless, slowly growing nodule, which may be solitary or appear as multiple lesions, which then arise metachronously and usually involve a particular anatomic region. Recurrence after surgical excision may occur in poorly circumscribed tumors. Malignancy is very rare.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-289685/","orphanet_url":"https://www.orpha.net/en/disease/detail/289685","names":{"fr":{"name":"Myopéricytome","definition":"Tumeur rare des tissus mous caractérisée par une lésion sous-cutanée bénigne composée de cellules d'aspect myoïde de forme ovale à fusiforme avec une tendance à se développer de manière circonférentielle périvasculaire. La tumeur se présente généralement sous la forme d'un nodule indolore, à croissance lente, qui peut être solitaire ou apparaître sous la forme de lésions multiples et se développant alors de manière métachrone et concernent généralement une région anatomique particulière. Une récidive après l'excision chirurgicale est possible dans les tumeurs mal circonscrites. La malignité est très rare."},"de":{"name":"Myoperizytom","definition":"Ein seltener Weichteiltumor, der eine gutartige subkutane Läsion darstellt, die aus ovalen bis spindelförmigen myoiden Zellen besteht und zu konzentrischem perivaskulärem Wachstum neigt. Der Tumor stellt sich in der Regel als schmerzloser, langsam wachsender Knoten dar, der solitär oder in Form multipler Läsionen auftreten kann, die sich metachron entwickeln und in der Regel eine bestimmte anatomische Region betreffen. Bei schlecht umschriebenen Tumoren kann es nach chirurgischer Exzision zu einem Rezidiv kommen. Malignität ist sehr selten."},"es":{"name":"Miopericitoma","definition":"Es un tumor de tejidos blandos poco frecuente caracterizado por una lesión benigna subcutánea compuesta por células de apariencia mioide entre ovaladas a fusiformes, con tendencia al crecimiento concéntrico perivascular. Por lo general, el tumor se presenta como un nódulo indoloro de crecimiento lento, pudiendo estar aislado o aparecer como múltiples lesionesmetacrónicas que afectan, por lo general, a una región anatómica concreta. Puede haber recurrencias tras la escisión quirúrgica en los tumores poco delimitados. El comportamiento maligno es muy poco frecuente."},"it":{"name":"Miopericitoma","definition":"Si tratta di un tumore raro dei tessuti molli, caratterizzato da una lesione sottocutanea benigna composta da cellule dall'aspetto mioide di forma ovale o fusiforme, con tendenza alla crescita perivascolare concentrica. In genere si manifesta sotto forma di nodulo indolore a crescita lenta isolato, o di lesioni multiple che insorgono in tempi successivi, interessando di solito una specifica regione anatomica. I tumori scarsamente circoscritti possono associarsi a recidive dopo l'asportazione chirurgica. L'evoluzione maligna è molto rara."},"nl":{"name":"Myopericytoom","definition":"Een zeldzame tumor van weke delen, gekarakteriseerd door een goedaardige, subcutane laesie bestaande uit ovale tot spoelvormige, myoïd-uitziende cellen met neiging tot concentrische perivasculaire groei. De tumor manifesteert zich meestal als een pijnloze, traaggroeiende nodulus, die solitair kan zijn of kan verschijnen als meervoudige laesies, die metachroon ontstaan en meestal een specifiek anatomisch gebied aantasten. Recidive na chirurgische excisie kan voorkomen bij weinig begrensde tumoren. Maligniteit is zeer zeldzaam."},"pt":{"name":"Miopericitoma"},"pl":{"name":"Miopericytoma"},"cs":{"name":"Myopericytom"},"uk":{"name":"Міоперицитома","definition":"A rare soft tissue tumor characterized by a benign subcutaneous lesion composed of oval-to-spindle shaped myoid appearing cells with a tendency for concentric perivascular growth. The tumor usually presents as a painless, slowly growing nodule, which may be solitary or appear as multiple lesions, which then arise metachronously and usually involve a particular anatomic region. Recurrence after surgical excision may occur in poorly circumscribed tumors. Malignancy is very rare."},"zh":{"name":"肌周细胞瘤","definition":"一种罕见的软组织肿瘤，其特征是良性皮下病变，由椭圆形至梭形的肌样细胞组成，血管周围有向心生长趋势。肿瘤常表现为同时发生的孤立性或多发性无痛性缓慢生长结节，常涉及特定解剖区域。手术切除后可在边界不清肿瘤处发生复发。恶性肿瘤少见。"},"tr":{"name":"Miyoperisitom"}},"xrefs":[{"source":"ICD-10","code":"D21.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017349","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D000077777","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1302808","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10083440","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":71209,"name":"Rare soft tissue tumor","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}