{"orpha":3398,"name":"Thymic epithelial neoplasm","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["TEN","Thymic epithelial tumor"],"definition":"Thymic epithelial neoplasms (TEN) are rare malignancies arising from the epithelium of the thymic gland. They comprise three sub-types: thymoma, thymic carcinoma, and thymic neuroendocrine carcinoma.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-3398/","orphanet_url":"https://www.orpha.net/en/disease/detail/3398","names":{"fr":{"name":"Néoplasie épithéliale thymique","synonyms":["TEN","Tumeur épithéliale thymique"],"definition":"Les néoplasies épithéliales thymiques sont des tumeurs malignes rares dérivées de l'épithélium de la glande thymique. Elles sont de trois types : le thymome, le carcinome thymique et le carcinome thymique neuro-endocrine."},"de":{"name":"Thymom, epitheliales","synonyms":["TEN"],"definition":"Die Thymusepithel-Neoplasmen (TEN) sind seltene Malignome, die im Epithel der Thymusdrüse entstehen. Zu ihnen gehören drei Untertypen: Thymom, Thymuskarzinom und neuroendokrines Tyhmuskarzinom (s. diese Termini)."},"es":{"name":"Neoplasia epitelial tímica","synonyms":["TEN","Tumor epitelial tímico"],"definition":"El pronóstico es desfavorable debido a la alta frecuencia de recurrencias y metástasis en pleura, pulmones, ganglios linfáticos, hueso, cerebro e hígado. La tasa de supervivencia a 5 años es del 35%."},"it":{"name":"Neoplasia epiteliale del timo","synonyms":["TEN","Tumore epiteliale del timo"]},"nl":{"name":"Epitheliaal neoplasma van thymus","synonyms":["Epitheliale tumor van thymus","TEN"],"definition":"Een zeldzame maligniteit die ontstaat uit epitheel van thymus. Drie subtypes worden onderscheiden: thymoom, thymuscarcinoom en neuro-endocrien carcinoom van thymus."},"pt":{"name":"Tumor epitelial do timo","definition":"As neoplasias epiteliais tímicas (TEN) são neoplasias malignas raras originando do epitélio da glândula tímica. Eles compreendem três sub-tipos: timoma, carcinoma tímico e carcinoma neuroendócrino tímico (ver estes termos)."},"pl":{"name":"Nowotwory nabłonkowe grasicy","synonyms":["Guz nabłonkowy grasicy"]},"cs":{"name":"Epiteliální nádor thymu","synonyms":["TEN"]},"uk":{"name":"Новоутворення епітелію тимуса","synonyms":["TEN","Епітеліальна пухлина вилочкової залози"],"definition":"Thymic epithelial neoplasms (TEN) are rare malignancies arising from the epithelium of the thymic gland. They comprise three sub-types: thymoma, thymic carcinoma, and thymic neuroendocrine carcinoma (see these terms)."},"zh":{"name":"胸腺上皮肿瘤","synonyms":["胸腺上皮肿瘤"]},"tr":{"name":"Timik epitel neoplazmı","synonyms":["TEN","Timik epitel tümörü"]}},"xrefs":[{"source":"MONDO","code":"0018079","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18079","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C536905","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1266101","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5201","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":100100,"name":"Thymic tumor","classifications":[202]}],"children":[{"orpha":99868,"name":"Thymic carcinoma","type":"Disease"},{"orpha":99869,"name":"Thymic neuroendocrine carcinoma","type":"Disease"},{"orpha":99867,"name":"Thymoma","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal recessive","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.17,"area":"Europe","status":"Validated","source":"[EXPERT]_RARECARE surveillance of rare cancers in Europe 2011[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.3,"area":"United States","status":"Validated","source":"22406029[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}