{"orpha":99915,"name":"Malignant granulosa cell tumor of the ovary","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Granulosa cell cancer","Granulosa cell malignant tumor"],"definition":"A rare malignant sex cord stromal tumor of ovary arising from the granulosa cells of the ovary, which occurs in peri and post menopausal women, and that presents with abnormal vaginal bleeding, abdominal pain and distension. The tumor is frequently unilateral, estrogen secreting, and has a slow natural history and a tendency to relapse long after the initial diagnosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99915/","orphanet_url":"https://www.orpha.net/en/disease/detail/99915","names":{"fr":{"name":"Tumeur maligne de la granulosa de l'ovaire","synonyms":["Cancer de la granulosa","Tumeur maligne de la granulosa"],"definition":"Tumeur stromale maligne rare des cordons sexuels et du stroma de l'ovaire se développant à partir des cellules de la granulosa ovarienne chez les femmes péri et post-ménopausées, se manifestant par des saignements vaginaux anormaux, des douleurs et une distension abdominales. Souvent unilatérale et sécrétant des oestrogènes, cette tumeur à histoire naturelle lente a tendance à récidiver longtemps après le diagnostic initial."},"de":{"name":"Maligner Granulosazelltumor des Ovars","synonyms":["Granulosazellkarzinom","Granulosazelltumor, maligner"],"definition":"Ein seltener maligner Keimstrang-Stromatumor des Eierstocks, der von den Granulosazellen des Eierstocks ausgeht, bei Frauen in und nach den Wechseljahren auftritt und sich durch abnormale vaginale Blutungen, Bauchschmerzen und Blähungen äußert. Der Tumor ist häufig einseitig, Östrogen-sekretierend, hat einen langsamen natürlichen Verlauf und neigt zu Rückfällen lange nach der Erstdiagnose."},"es":{"name":"Tumor maligno de células de la granulosa de ovario","synonyms":["Cáncer de células de la granulosa","Tumor maligno de células de la granulosa"],"definition":"El tumor ovárico maligno de células de la granulosa es un tumor del estroma de los cordones sexuales del ovario (véase este término) raro que se origina en las células de la granulosa del ovario; se observa en mujeres peri- y postmenopáusicas, y se presenta con hemorragia vaginal anómala, dolor abdominal y distensión. Con frecuencia se trata de un tumor unilateral, con secreción de estrógenos, y tiene una evolución natural lenta y con tendencia a recidivar mucho tiempo después del diagnóstico inicial, por lo que necesita un seguimiento prolongado."},"it":{"name":"Tumore ovarico maligno a cellule della granulosa","synonyms":["Carcinoma a cellule della granulosa","Tumore a cellule della granulosa","Tumore maligno delle cellule della granulosa"],"definition":"Il tumore ovarico maligno a cellule della granulosa è un tumore maligno raro dei cordoni sessuali e dello stroma (si veda questo termine) che origina dalle cellule della granulosa dell'ovaio. Interessa donne in peri- e post-menopausa e si manifesta con sanguinamento vaginale anomalo, dolore e distensione addominale. Il tumore è spesso monolaterale, estrogeno- secernente, ha una storia naturale lenta e la tendenza a recidivare molto tempo dopo la diagnosi iniziale, che richiede un follow-up a lungo termine."},"nl":{"name":"Maligne granulosaceltumor van ovarium","synonyms":["Granulosacelkanker","Kwaadaardige granulosaceltumor","Kwaadaardige granulosaceltumor van eierstok","Maligne granulosaceltumor"],"definition":"Een zeldzame maligne sex cord-stromale tumor van ovarium die ontstaat uit granulosacellen van ovarium, die optreedt bij peri- en postmenopauzale vrouwen, en die zich presenteert met abnormale vaginale bloeding en abdominale pijn en distensie. De tumor is vaak unilateraal, scheidt oestrogeen af, heeft een traag natuurlijk verloop en vertoont een neiging tot recidief lang na de initiële diagnose."},"pt":{"name":"Tumor de células granulosas"},"pl":{"name":"Złośliwy ziarniszczak jajnika","synonyms":["Ziarniszczak","Ziarniszczak złośliwy"],"definition":"Rzadki złośliwy nowotwór ze sznurów płciowych i zrębu jajnika, wywodzący się z komórek warstwy ziarnistej jajnika, który występuje u kobiet w okresie okołomenopauzalnym i po menopauzie i objawia się nieprawidłowym krwawieniem z pochwy, bólami brzucha i wzdęciem. Guz jest często jednostronny, wydziela estrogeny, naturalny przebieg choroby jest powolny, z tendencją do nawrotów długo po postawieniu wstępnej diagnozy."},"cs":{"name":"Maligní nádor ovaria z buněk granulózy","synonyms":["Rakovina z buněk granulózy","Maligní nádor z buněk granulózy"]},"uk":{"name":"Злоякісна гранульозоклітинна пухлина яєчника","synonyms":["Гранулезоклітинний рак","Гранулезоклітинна злоякісна пухлина"],"definition":"A rare malignant sex cord stromal tumor of ovary arising from the granulosa cells of the ovary, which occurs in peri and post menopausal women, and that presents with abnormal vaginal bleeding, abdominal pain and distension. The tumor is frequently unilateral, estrogen secreting, and has a slow natural history and a tendency to relapse long after the initial diagnosis."},"zh":{"name":"卵巢恶性颗粒细胞瘤","synonyms":["颗粒细胞恶性肿瘤"],"definition":"卵巢恶性颗粒细胞癌是一种罕见的恶性卵巢性索间质瘤，起源于卵巢颗粒细胞，发生于围绝经期和绝经后妇女，表现为异常阴道出血、腹痛和腹胀。肿瘤通常是单侧的，分泌雌激素的，自然病史缓慢，在最初诊断后很长一段时间内有复发的趋势，需要长时间的随访。"},"tr":{"name":"Overin maligant granüloza hücre tümörü","synonyms":["Granüloza hücreli kanser","Granüloza hücreli habis tümör"]}},"xrefs":[{"source":"ICD-10","code":"C56","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C73.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"148207042"},{"source":"MONDO","code":"0020541","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D006106","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0346175","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":"FOXL2","name":"forkhead box L2","gene_type":"gene with protein product","association":"Disease-causing somatic mutation(s) in","status":"Assessed","source":"19516027[PMID]","hgnc":"1092","ensembl":"ENSG00000183770","omim":"605597","loci":["3q22.3"]},{"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":["Multigenic/multifactorial"],"onset":["Adolescent","Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.12,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}