{"orpha":398971,"name":"Clear cell adenocarcinoma of the ovary","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Ovarian clear cell adenocarcinoma"],"definition":"A rare, malignant, epithelilal ovarian neoplasm, composed of clear, eosinophilic and hobnail cells displaying variable degrees of tubulocystic, papillary and solid histological patterns, macroscopically appearing as a typically unilateral mass in the ovary which ranges from solid to cystic. Patients are often diagnosed in early stages and usually present with pelvic pain and pressure, an abdominal mass and/or gastrointestinal problems, such as early satiety or bloating. Association with Lynch syndrome has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-398971/","orphanet_url":"https://www.orpha.net/en/disease/detail/398971","names":{"fr":{"name":"Adénocarcinome à cellules claires de l'ovaire","definition":"Un néoplasme épithélial ovarien, malin et rare. Il se compose de cellules « en clou de tapissier », de cellules éosinophiliques et de cellules claires, et présente des degrés variables de schémas histologiques solides, papillaires et tubulokystiques. Au niveau macroscopique, il se manifeste classiquement par une masse unilatérale solide à kystique, localisée au niveau de l'ovaire. Les patientes sont souvent diagnostiquées pendant les stades précoces de la maladie, et présentent habituellement une pression et une douleur pelviennes, une masse abdominale et/ou des troubles gastro-intestinaux, tels qu'une satiété précoce ou des ballonnements. Une association avec le syndrome de Lynch a été rapportée."},"de":{"name":"Klarzelliges Adenokarzinom des Ovars","synonyms":["Adenokarzinom, klarzelliges ovarielles"],"definition":"Eine seltene maligne epitheliale Neoplasie des Ovars, die aus klaren, eosinophilen und hobnagelförmigen Zellen besteht, die in unterschiedlichem Maße tubulozystische, papilläre und solide histologische Muster aufweisen und makroskopisch als typischerweise einseitige Masse im Ovar erscheinen, die von solide bis zystisch reicht. Die Patientinnen werden oft in frühen Stadien diagnostiziert und präsentieren sich meist mit Schmerzen und Druck im Beckenbereich, einer abdominalen Masse und/oder gastrointestinalen Problemen, wie frühzeitigem Sättigungsgefühl oder Blähungen. Es wurde über eine Assoziation mit dem Lynch-Syndrom berichtet."},"es":{"name":"Adenocarcinoma de células claras de ovario","synonyms":["Adenocarcinoma ovárico de células claras"],"definition":"Es una neoplasia ovárica epitelial, maligna y poco frecuente, compuesta por células en tachuela, claras y eosinofílicas, que muestran grados variables de patrones histológicos tubuloquísticos, papilares y sólidos, y que se presenta macroscópicamente como una masa típicamente unilateral en el ovario que varía de sólida a quística. Las pacientes se suelen diagnosticar en etapas tempranas y, por lo general, presentan dolor y presión pélvica, una masa abdominal y/o problemas gastrointestinales, tales como saciedad temprana o distensión abdominal. Se ha descrito la asociación con el síndrome de Lynch."},"it":{"name":"Adenocarcinoma a cellule chiare dell'ovaio","synonyms":["Adenocarcinoma ovarico a cellule chiare"],"definition":"L'adenocarcinoma dell'ovaio a cellule chiare è un tumore maligno raro dell'epitelio ovarico, composto da cellule chiare, eosinofile, con nucleo globoso, e quadri istologici tubulocistici, papillari e solidi di grado variabile; a livello macroscopico, l'adenocarcinoma si presenta come una massa, di solito monolaterale, localizzata nell'ovaio, di consistenza solida o cistica. Spesso la diagnosi viene posta precocemente. Le pazienti presentano dolore e tensione all'addome, associata alla presenza di una massa e/o a problemi gastrointestinali, come l'immediata sazietà o il gonfiore addominale. Può associarsi alla sindrome di Lynch."},"nl":{"name":"Heldercellig adenocarcinoom van ovarium","synonyms":["Heldercellig adenocarcinoom van eierstok","Ovarieel heldercellig adenocarcinoom"],"definition":"Een zeldzaam, maligne epitheliaal neoplasma van ovarium, bestaande uit heldere, eosinofiele en 'hobnail'-cellen die variabele mate van tubulocystische, papillaire en solide histologische patronen vertonen, en die er macroscopisch uitzien als een typische unilaterale massa in ovarium gaande van solide tot cystisch. Patiënten worden vaak in een vroeg stadium gediagnosticeerd en vertonen meestal pijn en druk in bekken, abdominale massa en/of gastro-intestinale problemen zoals vroegtijdige verzadiging of gezwollen abdomen. Er werd associatie met syndroom van Lynch gerapporteerd."},"pt":{"name":"Adenocarcinoma das células claras do ovário","synonyms":["Adenocarcinoma das células claras ováricas"]},"pl":{"name":"Gruczolakorak jasnokomórkowy jajnika","synonyms":["Gruczolakorakowiak jasnokomórkowy jajnika"]},"cs":{"name":"Světlobuněčný adenokarcinom ovaria","synonyms":["Ovariální světlobuněčný adenokarcinom"]},"uk":{"name":"Світлоклітинна аденокарцинома яєчника","synonyms":["Аденокарцинома яєчників з прозорими клітинами"],"definition":"A rare, malignant, epithelilal ovarian neoplasm, composed of clear, eosinophilic and hobnail cells displaying variable degrees of tubulocystic, papillary and solid histological patterns, macroscopically appearing as a typically unilateral mass in the ovary which ranges from solid to cystic. Patients are often diagnosed in early stages and usually present with pelvic pain and pressure, an abdominal mass and/or gastrointestinal problems, such as early satiety or bloating. Association with Lynch syndrome has been reported."},"zh":{"name":"卵巢透明细胞腺癌","synonyms":["卵巢透明细胞腺癌"],"definition":"卵巢透明细胞腺癌是一种少见的恶性上皮性卵巢肿瘤，由透明、嗜酸性和鞋甲细胞组成，表现为不同程度的管囊型、乳头状型和实体型组织学形态，肉眼上表现为典型的单侧卵巢肿块，范围从实性到囊性不等。患者通常在早期被诊断，通常表现为盆腔疼痛和压痛，腹部肿块和/或如早饱或腹胀胃肠道问题，。据报道本病与林奇综合征有关。"},"tr":{"name":"Overin berrak hücreli adenokarsinomu","synonyms":["Overin berrak hücreli adenokarsinomu"]}},"xrefs":[{"source":"ICD-10","code":"C56","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C73.00","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"315825558"},{"source":"MONDO","code":"0006045","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1518693","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"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.32,"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":0.112,"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":0.361,"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":0.201,"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":0.147,"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":0.165,"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":0.24,"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":0.359,"area":"Finland","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":0.182,"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":0.383,"area":"Iceland","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":0.395,"area":"Ireland","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":0.227,"area":"Italy","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":0.162,"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":0.166,"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":0.534,"area":"Malta","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":0.425,"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":0.118,"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":0.119,"area":"Portugal","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":0.273,"area":"Slovakia","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":0.388,"area":"Slovenia","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":0.343,"area":"Spain","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":0.282,"area":"Switzerland","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":0.342,"area":"Netherlands","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":0.451,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}