{"orpha":314478,"name":"Ovarian fibrothecoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Ovarian fibrothecoma is a rare, benign, sex cord-stromal neoplasm, with a typically unilateral location in the ovary, characterized by mixed features of both fibroma and thecoma. Patients may be asymptomatic or may present with pelvic/abdominal pain and/or distension and, occasionally, with post-menopausal bleeding. Large tumors (>10cm) are often associated with pleural effusion and ascites (the Meigs syndrome triad).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-314478/","orphanet_url":"https://www.orpha.net/en/disease/detail/314478","names":{"fr":{"name":"Fibrothécome ovarien","definition":"Le fibrothécome ovarien est un néoplasme rare et bénin des cordons sexuels et du stroma de l'ovaire, localisé habituellement de manière unilatérale au niveau de l'ovaire. Il présente des caractéristiques mixtes du fibrome et du thécome. Les patientes peuvent être asymptomatiques, ou présenter une douleur pelvienne/abdominale et/ou une distension, et parfois, un saignement après la ménopause. Les larges tumeurs (> 10cm) sont souvent associées à une effusion pleurale et à des ascites (triade du syndrome de Meigs)."},"de":{"name":"Fibrothekom des Ovars","definition":"Das Fibrothekom des Ovars ist ein seltener, gutartiger, Keimstrang-Stroma-Tumor mit typischerweise einseitiger Lokalisation im Ovar, der durch gemischte Merkmale von sowohl Fibrom als auch Thekom gekennzeichnet ist. Die Patientinnen können asymptomatisch sein oder sich mit Becken-/Bauchschmerzen und/oder Blähungen und gelegentlich mit postmenopausalen Blutungen vorstellen. Große Tumoren (>10 cm) sind oft mit Pleuraerguss und Aszites (Meigs-Syndrom-Trias) verbunden."},"es":{"name":"Fibrotecoma ovárico","definition":"El fibrotecoma ovárico es una neoplasia benigna poco frecuente del estroma del cordón sexual, con una localización típicamente unilateral en el ovario, caracterizada por rasgos mixtos tanto de fibroma como de tecoma. Las afectadas pueden ser asintomáticas o presentar dolor pélvico/abdominal y/o distensión y, ocasionalmente, con sangrado postmenopáusico. Con frecuencia, los tumores grandes (>10 cm) están asociados con derrame pleural y ascitis (triada del síndrome de Meig)."},"it":{"name":"Fibrotecoma ovarico","definition":"Il fibrotecoma ovarico è una neoplasia rara e benigna dello stroma e dei cordoni sessuali; di solito ha una localizzazione monolaterale all'interno dell'ovaio e associa le caratteristiche del fibroma e del tecoma. Le pazienti possono essere asintomatiche o presentare dolore pelvico/addominale e/o distensione e, raramente, emorragie post-menopausali. I tumori più grandi (>10 cm) spesso si associano a versamenti pleurici e ascite (triade della sindrome di Meigs)."},"nl":{"name":"Ovarieel fibrothecoom","definition":"Ovarieel fibrothecoom is een zeldzaam, goedaardig, sex cord-stromaal neoplasma met een kenmerkende unilaterale locatie in het ovarium en wordt gekarakteriseerd door een mengeling van kenmerken van zowel fibroom als thecoom. Patiënten kunnen asymptomatisch zijn of kunnen pijn aan het bekken/de buik en/of distensie vertonen, en in sommige gevallen ook postmenopauzale bloedingen. Grote tumoren (>10cm) zijn vaak geassocieerd met pleurale effusie en ascites (waterbuik) (de triade van het syndroom van Meig)."},"pt":{"name":"Fibrotecoma ovárico"},"pl":{"name":"Fibrotekoma jajnika"},"cs":{"name":"Ovariální fibrothékom"},"uk":{"name":"Фіброміома яєчників","definition":"Ovarian fibrothecoma is a rare, benign, sex cord-stromal neoplasm, with a typically unilateral location in the ovary, characterized by mixed features of both fibroma and thecoma. Patients may be asymptomatic or may present with pelvic/abdominal pain and/or distension and, occasionally, with post-menopausal bleeding. Large tumors (>10cm) are often associated with pleural effusion and ascites (the Meigs syndrome triad)."},"zh":{"name":"卵巢纤维膜瘤","definition":"卵巢纤维膜细胞瘤是一种罕见的良性性索间质肿瘤，位于单侧卵巢，具有纤维瘤和卵泡膜瘤的混合特征。患者可能无症状，或可能出现盆腔/腹痛和/或腹胀，偶尔伴有绝经后出血。大肿瘤（ inf 10cm）常伴有胸腹水（梅格斯综合征三联征）。"},"tr":{"name":"Over fibrotekom"}},"xrefs":[{"source":"ICD-10","code":"D27","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2F32.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1770220619"},{"source":"MONDO","code":"0017802","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707356","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":97293,"name":"Rare benign ovarian tumor","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-01-20","source":"16844153[PMID]_10193334[PMID]_11426907[PMID]_24670054[PMID]_8629912[PMID]_18501324[PMID]","terms":[{"hpo":"HP:0003270","label":"Abdominal distention","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000137","label":"Abnormality of the ovary","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100608","label":"Metrorrhagia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010618","label":"Ovarian fibroma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002586","label":"Peritonitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003117","label":"Abnormality of circulating hormone level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030126","label":"Abnormality of the endometrium","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006756","label":"Diffuse leiomyomatosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100244","label":"Fibrosarcoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008703","label":"Gonadal calcification","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001007","label":"Hirsutism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030088","label":"Increased serum testosterone level","frequency":"Occasional (29-5%)","criteria":null}]}}