{"orpha":314473,"name":"Ovarian fibroma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare benign ovarian tumor of sex cord / stromal origin characterized by an abdominal mass which may present with abdominal pain, distension, or menorrhagia, among others, or may also be asymptomatic. Association with ascites and hydrothorax is known as Meigs syndrome. The tumor can be solid and/or cystic in nature. Histologically it features bundles of spindle cells forming variable amounts of collagen, without cellular atypia or atypical mitoses.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-314473/","orphanet_url":"https://www.orpha.net/en/disease/detail/314473","names":{"fr":{"name":"Fibrome ovarien","definition":"Tumeur bénigne rare des cordons sexuels et du stroma de l'ovaire caractérisée par une masse abdominale qui peut se manifester par des douleurs et une distension abdominales, entre autres, ou par des ménorragies. Elle peut également être asymptomatique. L'association avec une ascite et un hydrothorax est connue sous le nom de syndrome de Meigs. La tumeur peut être de nature solide et/ou kystique. L'examen histologique fait apparaître des faisceaux de cellules fusiformes formant des quantités variables de fibres de collagène, sans atypies et sans mitoses atypiques."},"de":{"name":"Ovarialfibrom","definition":"Ein seltener gutartiger Tumor aus der Gruppe der ovariellen Keimstrang-Stromatumoren, der durch eine Gewebeverdichtung im Bauchraum gekennzeichnet ist, die unter anderem mit Bauchschmerzen, Blähungen oder Menorrhagie einhergehen oder auch asymptomatisch sein kann. Die Assoziation mit Aszites und Hydrothorax ist als Meigs-Syndrom bekannt. Der Tumor kann von solider und/oder zystischer Natur sein. Histologisch zeigt er Bündel von Spindelzellen, die unterschiedliche Mengen an Kollagen bilden, ohne zelluläre Atypien oder atypische Mitosen."},"es":{"name":"Fibroma ovárico","definition":"Es un tumor ovárico benigno poco frecuente de origen en el cordón sexual/ estroma caracterizado por una masa abdominal que puede presentarse con dolor abdominal, distensión o menorragia, entre otros, aunque también puede ser asintomático. La asociación con ascitis e hidrotórax se conoce como el síndrome de Meigs. El tumor puede ser de naturaleza sólida y/o quística. Histológicamente presenta haces de células fusiformes formando cantidades variables de colágeno, sin atipia celular ni mitosis atípica."},"it":{"name":"Fibroma ovarico","definition":"Si tratta di un tumore ovarico raro e benigno che origina dai cordoni sessuali/stroma, caratterizzato dalla presenza di una massa addominale; può esordire con dolore addominale, distensione o menorragia, ma le pazienti possono anche essere asintomatiche. Nel quadro della sindrome di Meigs si associa ad asciti e idrotorace. Il tumore può essere solido e/o cistico. Dal punto di vista istologico, è caratterizzato da fasci di cellule fusiformi che formano accumuli variabili di collagene, in assenza di cellule o di mitosi atipiche."},"nl":{"name":"Ovarieel fibroom","definition":"Een zeldzame, goedaardige tumor van ovarium van sex cord/stromale oorsprong, gekarakteriseerd door een abdominale massa die zich kan presenteren met onder meer abdominale pijn, distensie, of menorragie, of die ook asymptomatisch kan zijn. Associatie met ascites en hydrothorax is gekend als syndroom van Meigs. De tumor kan be solide en/of cystisch van aard zijn. Histologisch wordt het gekenmerkt door bundels van spoelcellen die variabele hoeveelheden collageen vormen, zonder cellulaire atypie of atypische mitosen."},"pt":{"name":"Fibroma ovárico"},"pl":{"name":"Włókniak jajnika"},"cs":{"name":"Ovariální fibrom"},"uk":{"name":"Фіброма яєчника","definition":"A rare benign ovarian tumor of sex cord / stromal origin characterized by an abdominal mass which may present with abdominal pain, distension, or menorrhagia, among others, or may also be asymptomatic. Association with ascites and hydrothorax is known as Meigs syndrome. The tumor can be solid and/or cystic in nature. Histologically it features bundles of spindle cells forming variable amounts of collagen, without cellular atypia or atypical mitoses."},"zh":{"name":"卵巢纤维瘤","definition":"一种罕见的性索/间质起源的良性卵巢肿瘤，以腹部肿块为特征，可伴有腹痛、腹胀或月经过多等症状，也可无症状。伴有腹水和胸腔积液则称为梅格斯综合征。肿瘤实质上可以是实性和/或囊性。组织学上表现为成束的纺锤形细胞，形成数量不等的胶原，无细胞异型性或不典型的有丝分裂。"},"tr":{"name":"Over fibromu"}},"xrefs":[{"source":"ICD-10","code":"D27","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2F32.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"871413134"},{"source":"MONDO","code":"0008168","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C562391","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0149951","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10064257","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":"3951494[PMID]_1256472[PMID]_24602407[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: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:0002671","label":"Basal cell carcinoma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008703","label":"Gonadal calcification","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030451","label":"Mesenteric cyst","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010603","label":"Odontogenic keratocysts of the jaw","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002586","label":"Peritonitis","frequency":"Frequent (79-30%)","criteria":null}]}}