{"orpha":66662,"name":"Extracutaneous mastocytoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare neoplastic disease characterized by a localized, unifocal, low-grade tumor composed of mature mast cells, without evidence of systemic mastocytosis or skin lesions. The tumor most commonly arises in the lung and shows a non-destructive growth pattern.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-66662/","orphanet_url":"https://www.orpha.net/en/disease/detail/66662","names":{"fr":{"name":"Mastocytome extra-cutané","definition":"Maladie néoplasique rare caractérisée par une tumeur de bas grade isolée et localisée, composée de mastocytes matures, en l'absence de signes de mastocytose systémique ou de lésions cutanées. La tumeur, non agressive, se développe aux dépends des poumons."},"de":{"name":"Mastozytom, extrakutanes","definition":"Eine seltene Neoplasie, die durch einen lokalisierten, unifokalen, niedriggradigen Tumor aus reifen Mastzellen gekennzeichnet ist, ohne Hinweise auf eine systemische Mastozytose oder Hautläsionen. Der Tumor entsteht am häufigsten in der Lunge und zeigt ein nicht destruktives Wachstumsmuster."},"es":{"name":"Mastocitoma extracutáneo","definition":"Es una enfermedad neoplásica poco frecuente caracterizada por un tumor de bajo grado, localizado, aislado, compuesto por mastocitos maduros, sin evidencia de mastocitosis sistémica o de lesiones cutáneas. El tumor se origina con mayor frecuencia en el pulmón, mostrando un patrón de crecimiento no destructivo."},"it":{"name":"Mastocitoma extracutaneo","definition":"Si tratta di una neoplasia rara, caratterizzata da un tumore localizzato, monofocale, di basso grado, composto da mastociti maturi, in assenza di mastocitosi sistemica o lesioni cutanee. In genere il tumore si localizza nei polmoni e presenta una crescita non distruttiva."},"nl":{"name":"Extracutaan mastocytoom","definition":"Een zeldzame neoplastische aandoening, gekarakteriseerd door een gelokaliseerde, unifocale, laaggradige tumor bestaande uit rijpe mestcellen, zonder bewijs van systemische mastocytose of huidlaesies. De tumor ontstaat doorgaans in long en toont een niet-destructief groeipatroon."},"pt":{"name":"Mastocitoma extra-cutâneo"},"pl":{"name":"Mastocytoza w narządach poza skórą"},"cs":{"name":"Mimokožní mastocytom"},"uk":{"name":"Позашкірна мастоцитома","definition":"A rare neoplastic disease characterized by a localized, unifocal, low-grade tumor composed of mature mast cells, without evidence of systemic mastocytosis or skin lesions. The tumor most commonly arises in the lung and shows a non-destructive growth pattern."},"zh":{"name":"皮肤外肥大细胞瘤"},"tr":{"name":"Ekstrakütanöz mastositoma"}},"xrefs":[{"source":"ICD-10","code":"C96.2","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2A21.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"29932455"},{"source":"MONDO","code":"0019025","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0272202","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":98292,"name":"Mastocytosis","classifications":[194,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":7.0,"area":"Worldwide","status":"Validated","source":"34233110[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"34233110[PMID]_ORPHANET"}],"phenotypes":null}