{"orpha":86855,"name":"Plasmacytoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Solitary plasmacytoma"],"definition":"Plasmacytoma is a localized mass of neoplastic monoclonal plasma cells that represents approximately 5% of all plasma cell neoplasms. There are two separate entities: primary plasmacytoma of the bone and extramedullary plasmacytoma of the soft tissues. Of the extramedullary plasmacytomas, 80% occur in the head and neck, usually in the upper respiratory tract. The median age at diagnosis is 50 years and the male to female ratio is 3:1. Long-term survival is possible following local radiotherapy, particularly for soft tissue presentations.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86855/","orphanet_url":"https://www.orpha.net/en/disease/detail/86855","names":{"fr":{"name":"Plasmocytome","synonyms":["Plasmacytome solitaire"],"definition":"Le plasmacytome est une masse tumorale localisée constituée de plasmocytes monoclonaux néoplasiques. Il représente environ 5% des néoplasies plasmocytaires. On distingue deux entités : le plasmacytome osseux primaire et le plasmacytome extra-médullaire des tissus mous. Parmi ces derniers, 80% se situent au niveau de la tête et du cou, généralement dans l'arbre respiratoire supérieur. L'âge moyen au diagnostic est de 50 ans et le ratio hommes/femmes est de 3:1. La survie à long terme est possible après une radiothérapie locale, surtout pour les plasmacytomes des tissus mous."},"de":{"name":"Plasmozytom","synonyms":["Plasmozytom, solitäres"],"definition":"Das Plasmozytom ist ein örtlich begrenzter Tumor aus monoklonalen Plasmazellen. Das Plasmozytom macht etwa 5% aller plasmazellulären Neoplasien aus und tritt in zwei Formen auf, als primäres Plasmozytom des Knochens und als extramedulläres Plasmozytom der Weichteile. Etwa 80% der extramedullären Plasmozytome treten an Kopf und Hals, meist in den oberen Atemwegen, auf. Das mittlere Alter bei der Diagnose ist 50 Jahre, das Geschlechtsverhältnis (m:w) ist 3:1. Lokale Strahlentherapie ermöglicht ein langes Überleben, besonders für Patienten mit Weichteiltumoren."},"es":{"name":"Plasmacitoma","synonyms":["Plasmacitoma solitario"],"definition":"Es un trastorno caracterizado por una masa localizada de células plasmáticas monoclonales neoplásicas que representa aproximadamente el 5% de todas las neoplasias de células plasmáticas. Comprende dos entidades diferenciadas: el plasmocitoma óseo primario y el plasmocitoma extramedular de tejidos blandos. Aproximadamente el 80% de los plasmocitomas extramedulares ocurre en la cabeza y el cuello, generalmente en el tracto respiratorio superior. La mediana de edad al momento del diagnóstico es de 50 años y el ratio hombre:mujer es de 3:1. La supervivencia a largo plazo es posible después de la radioterapia local, en particular para presentaciones en tejidos blandos."},"it":{"name":"Plasmocitoma","synonyms":["Plasmocitoma solitario"]},"nl":{"name":"Plasmacytoom","synonyms":["Solitair plasmacytoom"],"definition":"Plasmacytoom is een gelokaliseerde massa van neoplastische monoklonale plasmacellen, en vertegenwoordigt ongeveer 5% van alle neoplasmata van plasmacellen. Er zijn twee aparte entiteiten: primair plasmacytoom van bot en extramedullair plasmacytoom van weke delen. Van de extramedullaire plasmacytomen treedt 80% op in hoofd en hals, meestal in bovenste luchtwegen. De mediane leeftijd bij diagnose is bedraagt 50, en de man-vrouwverhouding is 3:1. Langdurige overleving is mogelijk na lokale radiotherapie, vooral bij presentatie in weke delen."},"pt":{"name":"Plasmocitoma"},"pl":{"name":"plazmocytoma","synonyms":["Odosobniony guz plazmocytowy"]},"cs":{"name":"Plazmocytom","synonyms":["Solitární plazmocytom"]},"uk":{"name":"Плазмоцитома","synonyms":["Солітарна плазмоцитома"],"definition":"Plasmacytoma is a localized mass of neoplastic monoclonal plasma cells that represents approximately 5% of all plasma cell neoplasms. There are two separate entities: primary plasmacytoma of the bone and extramedullary plasmacytoma of the soft tissues. Of the extramedullary plasmacytomas, 80% occur in the head and neck, usually in the upper respiratory tract. The median age at diagnosis is 50 years and the male to female ratio is 3:1. Long-term survival is possible following local radiotherapy, particularly for soft tissue presentations."},"zh":{"name":"浆细胞瘤","synonyms":["孤立性浆细胞瘤"]},"tr":{"name":"Plazmasitom","synonyms":["Soliter plazmasitom"]}},"xrefs":[{"source":"ICD-10","code":"C90.2","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C90.3","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A83.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1811140613"},{"source":"MONDO","code":"0005615","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D010954","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0032131","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"13446","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10035484","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":209016,"name":"Hematological disease associated with an acquired peripheral neuropathy","classifications":[181]},{"orpha":98282,"name":"Plasma cell tumor","classifications":[194,202,233]}],"children":[{"orpha":100022,"name":"Extramedullary soft tissue plasmacytoma","type":"Clinical subtype"},{"orpha":100021,"name":"Primary plasmacytoma of the bone","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.34,"area":"United States","status":"Validated","source":"19016727[PMID]_SEER Surveillance Epidemiology and End Results 1992-2004[OTHER]"}],"phenotypes":null}