{"orpha":99917,"name":"Theca steroid-producing cell malignant tumor of ovary, not further specified","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Theca (steroid-producing) cell cancer, not further specified"],"definition":"A rare malignant sex cord stromal tumor of ovary of unknown histological lineage, occurring in adult women, characterized, in most cases, by manifestations of androgen excess (hirsutism, hair loss, amenorrhea, or oligomenorrhea) and, occasionally, Cushing syndrome.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99917/","orphanet_url":"https://www.orpha.net/en/disease/detail/99917","names":{"fr":{"name":"Tumeur maligne à cellules stéroïdiennes de l'ovaire sans autre précision","synonyms":["Cancer à cellules stéroïdiennes sans autre précision"],"definition":"Tumeur maligne rare des cordons sexuels et du stroma de l'ovaire, dont l'origine histologique est inconnue, survenant chez les femmes adultes, caractérisée, dans la plupart des cas, par des symptômes d'hyperandrogénie (hirsutisme, perte de cheveux, aménorrhée ou oligoménorrhée) et, occasionnellement, par un syndrome de Cushing."},"de":{"name":"Steroid-produzierender maligner Thekazelltumor, nicht klassifizierter","synonyms":["Thekazellkarzinom (Steroid-produzierend), nicht klassifiziert"],"definition":"Ein seltener maligner Keimstrang-Stromatumor des Eierstocks unbekannter histologischer Abstammung, der bei erwachsenen Frauen auftritt und in den meisten Fällen durch Androgenüberschuss (Hirsutismus, Haarausfall, Amenorrhoe oder Oligomenorrhoe) und gelegentlich durch das Cushing-Syndrom gekennzeichnet ist."},"es":{"name":"Tumor maligno de las células esteroideas del ovario, sin especificar","synonyms":["Cáncer de células tecales (productoras de esteroides), sin más especificaciones"],"definition":"Es un tumor maligno poco frecuente del estroma de los cordones sexuales del ovario, de linaje histológico desconocido, que se observa en mujeres adultas y que se caracteriza, en la mayoría de los casos, por manifestaciones de exceso de andrógenos (hirsutismo, pérdida del cabello, amenorrea u oligomenorrea) y, ocasionalmente, síndrome de Cushing."},"it":{"name":"Tumore maligno delle cellule della teca dell'ovaio steroido-producenti, non altrimenti specificato","synonyms":["Cancro delle cellule della teca (steroido-producenti), non altrimenti specificato"],"definition":"Il tumore ovarico maligno a cellule steroidee, non altrimenti specificato, è un tumore ovarico maligno raro dei cordoni sessuali e dello stroma (si veda questo termine), dalle origini istologiche sconosciute, che si manifesta nelle donne adulte e si caratterizza, nella maggior parte dei casi, per i segni clinici dell'iperandrogenismo (irsutismo, perdita di capelli, amenorrea, o oligomenorrea) e, raramente, per la sindrome di Cushing (si veda questo termine)."},"nl":{"name":"Maligne steroïdeproducerende thecaceltumor van ovarium, niet verder gespecifieerd","synonyms":["Kwaadaardige steroïdeproducerende thecaceltumor van eierstok, niet verder gespecifieerd","Thecacelkanker (steroïdeproducerend), niet verder gespecifieerd"],"definition":"Een zeldzame maligne sex cord-stromale tumor van ovarium met onbekende histologische oorsprong, die optreedt bij volwassen vrouwen en in de meeste gevallen gekenmerkt wordt door manifestaties van overmaat aan androgeen (hirsutisme, haarverlies, amenorroe of oligomenorroe) en soms syndroom van Cushing."},"pt":{"name":"Tumor de células da teca (produtor de esteróides), não especificado"},"pl":{"name":"Theca steroid-producing cell malignant tumor of ovary, not further specified","synonyms":["Theca (steroid-producing) cell cancer, not further specified"],"definition":"Rzadki złośliwy nowotwór ze sznurów płciowych i zrębu jajnika o nieznanym pochodzeniu histologicznym, występujący u dorosłych kobiet, charakteryzujący się w większości przypadków objawami hiperandrogenizmu (hirsutyzm, wypadanie włosów, brak miesiączki lub skąpe miesiączki) i sporadycznie zespołem Cushinga."},"cs":{"name":"Dále nespecifikovaný maligní nádor ovaria s produkcí steroidů z thekálních buněk","synonyms":["Dále nespecifikovaná rakovina z thekálních buněk produkujících steroidy"]},"uk":{"name":"Злоякісна пухлина яєчника з стероїдпродукуючими клітинами Тека, не уточнена","synonyms":["Тека (стероїдопродукуючий) клітинний рак, не уточнено"],"definition":"A rare malignant sex cord stromal tumor of ovary of unknown histological lineage, occurring in adult women, characterized, in most cases, by manifestations of androgen excess (hirsutism, hair loss, amenorrhea, or oligomenorrhea) and, occasionally, Cushing syndrome."},"zh":{"name":"卵巢激素合成性卵泡膜细胞恶性肿瘤未指定类型","synonyms":["卵泡膜(产生类固醇)细胞癌未指定型"],"definition":"卵巢恶性类固醇细胞瘤是一种罕见的卵巢恶性性索间质瘤(见该词条)，其组织谱系不明，发生在成年女性，大多数病例以雄激素过多(多毛、脱发、闭经或月经过少)为特征，偶尔还会出现库欣综合征(见该词条)。"},"tr":{"name":"Teka steroid üreten habis hücreli over tümörü-tam tanımlanmamış","synonyms":["Teka (steroid üreten) hücreli kanser, daha fazla belirtilmemiş"]}},"xrefs":[{"source":"ICD-10","code":"C56","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C73.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"548776312"},{"source":"MONDO","code":"0020543","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4274494","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":35808,"name":"Malignant sex cord stromal tumor of ovary","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}