{"orpha":99916,"name":"Malignant Sertoli-Leydig cell tumor of the ovary","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Androblastoma","Arrhenoblastoma","Ovarian Sertoli-Leydig cell cancer","Ovarian malignant Sertoli-Leydig cell tumor","Virilizing ovarian tumor"],"definition":"A rare malignant sex cord stromal tumor of ovary occuring typically in young women and characterized by manifestations of androgen excess (hirsutism, hair loss, amenorrhea, or oligomenorrhea), when functional.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99916/","orphanet_url":"https://www.orpha.net/en/disease/detail/99916","names":{"fr":{"name":"Tumeur maligne de Sertoli-Leydig de l'ovaire","synonyms":["Androblastome","Arrhénoblastome","Cancer de Sertoli-Leydig de l'ovaire","Tumeur ovarienne maligne de Sertoli-Leydig","Tumeur virilisante de l'ovaire"],"definition":"Tumeur maligne rare des cordons sexuels et du stroma de l'ovaire survenant généralement chez les jeunes femmes, caractérisée par des signes cliniques d'une hyperandrogénie (hirsutisme, perte de cheveux, aménorrhée ou oligoménorrhée), lorsqu'elle est fonctionnelle."},"de":{"name":"Maligner Sertoli-Leydig-Zell-Tumor des Ovars","synonyms":["Androblastom","Arrhenoblastom","Ovarialtumor, virilisierender","Sertoli-Leydig Zell-Tumor, ovarieller","Sertoli-Leydig-Zell-Tumor, maligner ovarieller","Sertoli-Zell-Karzinom des Ovars"],"definition":"Ein seltener maligner Keimstrang-Stromatumor des Eierstocks, der typischerweise bei jungen Frauen auftritt und durch Androgenüberschuss (Hirsutismus, Haarausfall, Amenorrhoe oder Oligomenorrhoe) gekennzeichnet ist, wenn er funktionsfähig ist."},"es":{"name":"Tumor maligno de las células de Sertoli-Leydig de ovario","synonyms":["Androblastoma","Arrenoblastoma","Cáncer ovárico de células de Sertoli-Leydig","Tumor ovárico maligno de células de Sertoli-Leydig","Tumor ovárico virilizante"],"definition":"Es un tumor maligno poco frecuente del estroma de los cordones sexuales del ovario que se observa principalmente en mujeres jóvenes y que se caracteriza por manifestaciones de exceso de andrógenos (hirsutismo, pérdida del cabello, amenorrea u oligomenorrea) cuando es funcionante."},"it":{"name":"Tumore maligno dell'ovaio a cellule del Sertoli-Leydig","synonyms":["Androblastoma","Arrenoblastoma","Cancro ovarico a cellule del Sertoli-Leydig","Tumore ovarico maligno a cellule del Sertoli-Leydig","Tumore ovarico virilizzante"],"definition":"Il tumore ovarico maligno a cellule del Sertoli-Leydig è un tumore ovarico maligno raro dei cordoni sessuali e dello stroma (si veda questo termine), che si manifesta tipicamente nelle donne giovani e si caratterizza per i segni clinici dell'eccesso di androgeni (irsutismo, perdita di capelli, amenorrea od oligomenorrea)."},"nl":{"name":"Maligne Sertoli-Leydigceltumor van ovarium","synonyms":["Androblastoom","Arrhenoblastoom","Kwaadaardige Sertoli-Leydigceltumor van eierstok","Ovariële Sertoli-Leydigcelkanker","Ovariële maligne Sertoli-Leydigceltumor","Viriliserende ovariumtumor"],"definition":"Een zeldzame maligne sex cord-stromale tumor van ovarium, die doorgaans optreedt bij jonge vrouwen en wanneer hij functioneel is gekenmerkt wordt door manifestaties van overmaat aan androgeen (hirsutisme, haarverlies, amenorroe of oligomenorroe)."},"pt":{"name":"Tumor ovárico de células de Sertoli-Leydig"},"pl":{"name":"Złośliwy guz jajnika z komórek Sertoliego i Leydiga","synonyms":["Androblastoma","Arrenoblastoma/Jądrzak","Nowotwór jajnika z komórek Sertoliego i Leydiga","Wirilizujący guz jajnika"],"definition":"Rzadki złośliwy nowotwór ze sznurów płciowych i zrębu jajnika, typowo występujący u młodych kobiet i charakteryzujący się objawami związanymi z hiperandrogenizmem (hirsutyzm, wypadanie włosów, brak miesiączki lub skąpe miesiączki), gdy jest hormonalnie czynny."},"cs":{"name":"Maligní nádor ovaria ze Sertoliho-Leydigových buněk","synonyms":["Androblastom","Arhenoblastom","Rakovina vaječníku ze Sertoliho-Leydigových buněk","Maligní nádor ze Sertoliho-Leydigových buněk","Virilizující nádor vaječníků"]},"uk":{"name":"Злоякісна Сертолі-Лейдіга-клітинна пухлина яєчника","synonyms":["Андробластома","Артенобластома","Злоякісна клітинна пухлина яєчників Сертолі-Лейдіга","Клітинний рак яєчників Сертолі-Лейдіга","Вірилізуюча пухлина яєчника"],"definition":"A rare malignant sex cord stromal tumor of ovary occuring typically in young women and characterized by manifestations of androgen excess (hirsutism, hair loss, amenorrhea, or oligomenorrhea), when functional."},"zh":{"name":"卵巢恶性支持-间质细胞瘤","synonyms":["卵巢恶性支持间质细胞肿瘤"],"definition":"卵巢恶性支持-间质细胞癌是一种罕见的卵巢恶性性索间质肿瘤(见该词条)，多见于年轻女性，其特征是功能性雄激素过多症状(多毛、脱发、闭经或月经过少)。"},"tr":{"name":"Overin habis Sertoli-Leydig hücre tümörü","synonyms":["Androblastoma","Arenoblastom","Virilize yumurtalık tümörü","Yumurtalık Sertoli-Leydig hücre kanseri","Yumurtalık habis Sertoli-Leydig hücre tümörü"]}},"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":"1092610858"},{"source":"MONDO","code":"0020542","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5574973","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5495","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":[{"symbol":"DICER1","name":"dicer 1, ribonuclease III","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"24761742[PMID]_21205968[PMID]_21882293[PMID]","hgnc":"17098","ensembl":"ENSG00000100697","omim":"606241","loci":["14q32.13"]}],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult","Childhood","Elderly"],"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}