{"orpha":35808,"name":"Malignant sex cord stromal tumor of ovary","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["Malignant ovarian SCST","Malignant ovarian sex cord-stromal tumor"],"definition":"Malignant sex cord stromal tumor (SCST) of ovary is a rare ovarian cancer arising from granulosa, theca, sertoli and leydig cells or stromal fibroblasts, occurring at any age and presenting with abdominal or pelvic mass, and characterized (with the exception of fibroma) by the production of sex steroids resulting in manifestations of hormone excess, with a relatively favorable prognosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-35808/","orphanet_url":"https://www.orpha.net/en/disease/detail/35808","names":{"fr":{"name":"Tumeur maligne des cordons sexuels et du stroma de l'ovaire","synonyms":["SCST ovarienne maligne","Tumeur ovarienne maligne stromale des cordons sexuels"],"definition":"La tumeur maligne des cordons sexuels et du stroma de l'ovaire (SCST ovarienne maligne) est un cancer rare de l'ovaire, issu des cellules de la granulosa, de la thèque, de Sertoli et de Leydig ou des fibroblastes stromaux, survenant à n'importe quel âge et se manifestant par une masse abdominale ou pelvienne. A l'exception du fibrome, elle se caractérise par la production de stéroïdes sexuels, ce qui conduit à des manifestations d'excès d'hormone, avec un pronostic relativement favorable."},"de":{"name":"Maligne Keimstrang-Stroma-Tumoren des Ovars","synonyms":["OSCST"]},"es":{"name":"Tumor maligno del estroma de los cordones sexuales de los ovarios","synonyms":["SCST maligno ovárico","Tumor estromal maligno de los cordones sexuales ováricos"],"definition":"El tumor maligno del estroma de los cordones sexuales (SCST) de los ovarios es un cáncer ovárico raro (véase este término) que surge de la granulosa, la teca, las células de Sertoli y las células de Leydig o los fibroblastos del estroma. Se presenta a cualquier edad como un tumor abdominal o pélvico y se caracteriza (a excepción del fibroma) por la producción de esteroides sexuales que provocan manifestaciones de exceso hormonal, con un pronóstico relativamente favorable."},"it":{"name":"Tumore maligno dello strome e dei cordoni sessuali dell'ovaio","synonyms":["Cancro ovarico dello stroma e dei cordoni sessuali","SCST ovarico maligno","Tumore ovarico maligno dello stroma e dei cordoni sessuali"],"definition":"Il tumore maligno dello stroma e dei cordoni sessuali (SCST) dell'ovaio è un cancro ovarico raro (si veda questo termine) che insorge dalle cellule della granulosa, della teca, del Sertoli e dalle cellule di Leydig o dai fibroblasti stromali, con un esordio a tutte le età, sotto forma di massa addominale o pelvica e la produzione (fatta eccezione per il fibroma) di livelli aumentati di steroidi sessuali ed una prognosi relativamente sfavorevole."},"nl":{"name":"Maligne sex cord-stromale tumor van ovarium","synonyms":["Kwaadaardige sex cord-stromale tumor van eierstok","Maligne ovariële SCST","Maligne ovariële sex cord-stromale tumor"],"definition":"Maligne sex cord-stromale tumor (SCST) van het ovarium is een zeldzame ovariumkanker (zie deze term) die ontstaat uit granulosa-, theca-, Sertoli-, of Leydigcellen of stromale fibroblasten en kan voorkomen op elke leeftijd. De ziekte uit zich met zwelling in de buik of het bekken, wordt gekarakteriseerd (met uitzondering van fibroom) door de productie van geslachtshormonen met kenmerken van een teveel aan hormonen tot gevolg, en heeft een relatief gunstige prognose."},"pt":{"name":"Tumor maligno do estroma dos cordões sexuais do ovário","synonyms":["SCST ovárico maligno"]},"pl":{"name":"Złośliwy guz jajnika z komórek sznurów płciowych i podścieliska","synonyms":["Złośliwy SCST jajnika"]},"cs":{"name":"Maligní sex-cord stromální tumor ovaria","synonyms":["Maligní ovariální SCST","Maligní ovariální sex cord-stromální tumor"]},"uk":{"name":"Злоякісна стромальна пухлина статевого канатика яєчника","synonyms":["Злоякісний рак яєчника","Злоякісна пухлина статевого канатика-строми яєчника"],"definition":"Malignant sex cord stromal tumor (SCST) of ovary is a rare ovarian cancer (see this term) arising from granulosa, theca, sertoli and leydig cells or stromal fibroblasts, occurring at any age and presenting with abdominal or pelvic mass, and characterized (with the exception of fibroma) by the production of sex steroids resulting in manifestations of hormone excess, with a relatively favorable prognosis."},"zh":{"name":"恶性卵巢性索间质肿瘤","synonyms":["恶性卵巢性索-间质瘤"]},"tr":{"name":"Yumurtalıkların habis cinsiyet kord stromal tümörü","synonyms":["Habis yumurtalık SCST","Habis yumurtalık cinsiyet kord stromal tümörü"]}},"xrefs":[{"source":"MONDO","code":"0018172","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18172","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1334609","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":398940,"name":"Malignant non-epithelial tumor of ovary","classifications":[202,205]}],"children":[{"orpha":99914,"name":"Gynandroblastoma","type":"Disease"},{"orpha":99916,"name":"Malignant Sertoli-Leydig cell tumor of the ovary","type":"Disease"},{"orpha":99915,"name":"Malignant granulosa cell tumor of the ovary","type":"Disease"},{"orpha":99917,"name":"Theca steroid-producing cell malignant tumor of ovary, not further specified","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.85,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.13,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.1,"area":"United States","status":"Validated","source":"15863154[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.05,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.043,"area":"Iceland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.074,"area":"Ireland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.069,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.094,"area":"Malta","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.045,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.063,"area":"Slovenia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.039,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.073,"area":"Switzerland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.093,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.089,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.133,"area":"Austria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.102,"area":"Belgium","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.367,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.152,"area":"Croatia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.314,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.296,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.135,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.401,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.236,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.12,"area":"Norway","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.232,"area":"Poland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.212,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}