{"orpha":213792,"name":"Adenosarcoma of the cervix uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Cervical adenosarcoma"],"definition":"A rare subtype of malignant mixed epithelial and mesenchymal tumor composed of benign or mildly atypical glandular elements and a surrounding low-grade malignant stroma, often containing heterologous elements, such as areas of sex-cord-like or smooth muscle differentiation. It usually presents with vaginal bleeding or discharge, lower abdominal pain and/or a cervical mass or polyp. The tumor may arise from pre-existing endometriosis and patients may have a history of recurrent cervical polyps.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213792/","orphanet_url":"https://www.orpha.net/en/disease/detail/213792","names":{"fr":{"name":"Adénosarcome du col de l'utérus","synonyms":["Adénosarcome cervical"],"definition":"Un sous-type rare de tumeur maligne mixte, épithéliale et mésenchymateuse. Il se compose d'éléments glandulaires bénins ou légèrement atypiques, et d'un stroma environnant malin de bas grade, contenant souvent des éléments hétérologues, tels que des zones de différenciation en muscle lisse ou en une structure ressemblant aux cordons sexuels. Il se présente généralement par un saignement ou une perte vaginale, une douleur dans le bas ventre et/ou une masse ou un polype cervical. La tumeur peut tirer son origine d'une endométriose préexistante, et les patientes peuvent avoir des antécédents de polypes cervicaux récurrents."},"de":{"name":"Adenosarkom der Cervix uteri","synonyms":["Adenosarkom, zervikales"],"definition":"Ein seltener Subtyp eines malignen gemischten epithelialen und mesenchymalen Tumors, der aus gutartigen oder leicht atypischen Drüsenelementen und einem umgebenden niedriggradig malignen Stroma besteht, das oft heterologe Elemente enthält, wie z. B. Bereiche mit keimstrangartiger oder glatter Muskeldifferenzierung. In der Regel treten vaginale Blutungen oder Ausfluss, Unterleibsschmerzen und/oder eine zervikale Masse oder ein Polyp auf. Der Tumor kann aus einer vorbestehenden Endometriose entstehen und die Patientinnen können eine Vorgeschichte mit rezidivierenden Zervixpolypen haben."},"es":{"name":"Adenosarcoma de cuello de útero","synonyms":["Adenosarcoma cervical"],"definition":"Es un subtipo poco frecuente de tumor mixto maligno epitelial y mesenquimatoso compuesto por elementos glandulares benignos o levemente atípicos y un estroma maligno circundante de grado bajo, que a menudo contiene elementos heterólogos, tales como áreas de diferenciación del músculo liso o similar al cordón sexual. Generalmente se presenta con sangrado o secreción vaginal, dolor abdominal inferior y/o una masa o pólipo cervical. El tumor puede surgir de una endometriosis preexistente y las afectadas pueden tener un historial de pólipos cervicales recurrentes."},"it":{"name":"Adenosarcoma della cervice uterina","synonyms":["Adenosarcoma cervicale"],"definition":"L'adenosarcoma della cervice uterina è un sottotipo raro di tumore maligno misto epiteliale e mesenchimale, composto da elementi ghiandolari benigni o lievemente atipici e da uno stroma circostante a basso grado di malignità, spesso contenente elementi eterologhi, come aree differenziate nella muscolatura liscia o in una struttura simile ai cordoni sessuali. Di solito esordisce con emorragie o perdite vaginali, dolore localizzato nella parte inferiore dell'addome e/o con la presenza di una massa o un polipo cervicale. Il tumore può originare su una preesistente endometriosi e le pazienti possono avere un'anamnesi positiva per poliposi cervicale."},"nl":{"name":"Adenosarcoom van cervix uteri","synonyms":["Adenosarcoom van baarmoederhals","Cervicaal adenosarcoom"],"definition":"Een zeldzaam subtype van maligne gemengde epitheliale en mesenchymale tumor bestaande uit goedaardige of mild atypische glandulaire elementen en een omringend laaggradig maligne stroma, vaak met heterologe elementen, zoals gebieden met sex cord-achtige of gladde spier differentiatie. Het manifesteert zich meestal met vaginale bloeding of afscheiding, pijn in de onderbuik en/of een cervicale massa of poliep. De tumor kan voortkomen uit reeds aanwezige endometriose en patiënten hebben mogelijk een voorgeschiedenis met terugkerende cervicale poliepen."},"pt":{"name":"Adenossarcoma do colo do útero"},"pl":{"name":"Gruczolakomięsak szyjki macicy","synonyms":["Gruczolakomięsak szyjki"]},"cs":{"name":"Adenosarkom hrdla děložního","synonyms":["Cervikální adenosarkom"]},"uk":{"name":"Аденосаркома шийки матки","synonyms":["Аденосаркома шийки матки"],"definition":"A rare subtype of malignant mixed epithelial and mesenchymal tumor composed of benign or mildly atypical glandular elements and a surrounding low-grade malignant stroma, often containing heterologous elements, such as areas of sex-cord-like or smooth muscle differentiation. It usually presents with vaginal bleeding or discharge, lower abdominal pain and/or a cervical mass or polyp. The tumor may arise from pre-existing endometriosis and patients may have a history of recurrent cervical polyps."},"zh":{"name":"子宫颈腺肉瘤","synonyms":["宫颈腺肉瘤"],"definition":"一种罕见的上皮间质混合恶性肿瘤，由良性或轻度不典型腺体成分和周围的低度恶性间质组成，常含有异源成分，如性索样或平滑肌分化区域。本病通常表现为阴道出血或分泌物、下腹痛和/或宫颈肿块或息肉。肿瘤可能起源于已有的子宫内膜异位症，患者可能有复发性宫颈息肉的病史。"},"tr":{"name":"Serviks uteri adenosarkomu","synonyms":["Servikal adenosarkom"]}},"xrefs":[{"source":"ICD-10","code":"C53.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C53.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C53.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C77.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1761315829"},{"source":"MONDO","code":"0002876","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1516426","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":213782,"name":"Malignant mixed epithelial and mesenchymal tumor of cervix uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_19685064[PMID]"}],"phenotypes":null}