{"orpha":454723,"name":"Endometrioid carcinoma of ovary","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare malignant epithelial tumor of ovary characterized by confluent or cribriform proliferations of round, oval, or tubular glands, typically lined by stratified non-mucin-containing epithelium with well-defined luminal margins. Squamous differentiation, secretory changes, oxyphilic variants, sex cord-stromal type patterns, or sertoliform endometrioid carcinomas may occur. Patients most commonly present in the sixth decade of life, either with a pelvic mass with or without pain, or without any symptoms. The tumor may be bilateral and is frequently associated with endometriosis and/or endometrial carcinoma.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-454723/","orphanet_url":"https://www.orpha.net/en/disease/detail/454723","names":{"fr":{"name":"Adénocarcinome endométrioïde de l'ovaire","definition":"Tumeur épithéliale maligne rare de l'ovaire caractérisée par des proliférations confluentes ou cribriformes de glandes de forme ronde, ovale ou tubulaire, typiquement tapissées d'un épithélium stratifié ne contenant pas de mucine, aux marges luminales bien définies. On peut observer des carcinomes endométrioïdes à cellules squameuses, de variante sécrétoire, de variantes oxyphiles, du stroma et des cordons sexuels. Généralement, les patientes présentent dans la sixième décennie de vie une masse pelvienne accompagnée ou non de douleurs, ou alors ne présentent pas de symptômes. Souvent associée à une endométriose et/ou un carcinome de l'endomètre, la tumeur peut être bilatérale."},"de":{"name":"Endometrioides Ovarialkarzinom","synonyms":["Endometriumkarzinom des Ovars"],"definition":"Ein seltener maligner Epitheltumor des Ovars, der durch konfluierende oder rippenartige Wucherungen runder, ovaler oder tubulärer Drüsen gekennzeichnet ist, die typischerweise von einem schichtförmigen, nicht muzinösen Epithel mit scharf abgegrenzten Luminalrändern ausgekleidet sind. Plattenepitheliale Differenzierungen, sekretorische Veränderungen, oxyphile Varianten, sex cord-stromale Muster oder sertoliforme endometrioide Karzinome können auftreten. Die Patientinnen stellen sich meist im sechsten Lebensjahrzehnt mit einer Gewebsverdichtung im Beckenbereich mit oder ohne Schmerzen oder ohne Symptome vor. Der Tumor kann beidseitig auftreten und ist häufig mit einer Endometriose und/oder einem Endometriumkarzinom assoziiert."},"es":{"name":"Carcinoma endometrioide de ovario","definition":"Es un tumor epitelial maligno de ovario poco frecuente caracterizado por proliferaciones confluentes o cribiformes de glándulas redondeadas, ovaladas o tubulares, típicamente revestidas por epitelio estratificado sin mucina y márgenes luminales bien definidos. Puede producirse una diferenciación escamosa, cambios secretores, variantes oxifílicas, patrones estromales tipo cordones sexuales o carcinomas endometrioides sertoliformes. Las pacientes se presentan con mayor frecuencia en la sexta década de la vida, ya sea con una masa pélvica con o sin dolor, o sin ningún síntoma. El tumor puede ser bilateral y se asocia frecuentemente con endometriosis y/o carcinoma de endometrio."},"it":{"name":"Carcinoma endometrioide dell'ovaio","definition":"Si tratta di una neoplasia epiteliale maligna rara dell'ovaio, caratterizzata da proliferazioni confluenti o cribriformi di ghiandole rotonde, ovali o tubulari, di solito rivestite da un epitelio stratificato non contenente mucina con margini luminali ben definiti. Le pazienti possono presentare differenziazione squamosa, alterazioni secretorie, varianti ossifile, tipologia stromale/dei cordoni sessuali o carcinomi endometrioidi sertoliformi. Di solito la malattia esordisce nella sesta decade di vita sotto forma di una massa pelvica dolorosa o indolore, ma le pazienti possono anche essere asintomatiche. Il tumore può essere bilaterale e spesso si associa all'endometriosi e/o al carcinoma dell'endometrio."},"nl":{"name":"Endometrioïd carcinoom van ovarium","definition":"Een zeldzame maligne epitheliale tumor van ovarium, gekarakteriseerd door confluente of cribriforme proliferatie van ronde, ovale of tubulaire klieren, doorgaans omzoomd door gelaagd epitheel dat geen mucine bevat en goed gedefinieerde luminale randen vertoond. Squameuze differentiatie, secretoire veranderingen, oxyfiele varianten, 'sex cord'-stroma-type patronen, of sertoliform endometrioïd carcinoom kan voorkomen. Patiënten presenteren zich doorgaans in het zesde levensdecennium, ofwel met een pelviene massa met of zonder pijn, ofwel zonder enige symptomen. De tumor kan bilateraal zijn en is vaak geassocieerd met endometriose en/of carcinoom van endometrium."},"pt":{"name":"Carcinoma endometrioide do ovário"},"pl":{"name":"Endometrialny rak jajnika"},"cs":{"name":"Endometroidní karcinom ovaria"},"uk":{"name":"Ендометріоїдна карцинома яєчника","definition":"A rare malignant epithelial tumor of ovary characterized by confluent or cribriform proliferations of round, oval, or tubular glands, typically lined by stratified non-mucin-containing epithelium with well-defined luminal margins. Squamous differentiation, secretory changes, oxyphilic variants, sex cord-stromal type patterns, or sertoliform endometrioid carcinomas may occur. Patients most commonly present in the sixth decade of life, either with a pelvic mass with or without pain, or without any symptoms. The tumor may be bilateral and is frequently associated with endometriosis and/or endometrial carcinoma."},"zh":{"name":"子宫内膜样卵巢癌"},"tr":{"name":"Yumurtalıkta endometrioid karsinom"}},"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":"1798859381"},{"source":"MONDO","code":"0006335","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"6335","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0346163","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10073269","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":398934,"name":"Malignant epithelial tumor of ovary","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.51,"area":"Korea, Republic of","status":"Validated","source":"26463436[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.81,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}