{"orpha":213823,"name":"Adenoid cystic carcinoma of the cervix uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Cervical adenoid cystic carcinoma"],"definition":"A rare, highly aggressive uterine cancer, macroscopically appearing as an irregular, slow-growing, non-friable, polypoid mass on the uterine cervix and histologically showing a pseudoglandular or cribriform growth pattern. It presents with vaginal bleeding and discharge and abdominal or pelvic pain. The tumor is highly infiltrative, often associated with vascular, lymphatic and perineural invasion, with subsequent haematogenous spread and early recurrence.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213823/","orphanet_url":"https://www.orpha.net/en/disease/detail/213823","names":{"fr":{"name":"Carcinome adénoïde kystique du col de l'utérus","synonyms":["Carcinome adénoïde kystique cervical"],"definition":"Un cancer utérin rare, très agressif, se manifestant sur le plan macroscopique par une masse polypoïde irrégulière, non friable, lentement progressive située au niveau du col de l'utérus. Sur le plan histologique, il présente par un schéma de croissance pseudo-glandulaire ou cribriforme. Il se manifeste par un saignement et des pertes vaginales, ainsi qu'une masse abdominale ou pelvienne. La tumeur est très infiltrante, souvent associée à une invasion vasculaire, lymphatique et périneurale, avec une dispersion hématogène et une récurrence précoce."},"de":{"name":"Adenoides zystisches Karzinom der Cervix uteri","synonyms":["Zervixkarzinom, adenoides zystisches"],"definition":"Ein seltenes, hochaggressives Uteruskarzinom, das makroskopisch als unregelmäßige, langsam wachsende, nicht brüchige, polypoide Masse am Gebärmutterhals auftritt und histologisch ein pseudoglanduläres oder kribriformes Wachstumsmuster zeigt. Er präsentiert sich mit vaginalen Blutungen und Ausfluss sowie abdominalen oder pelvinen Schmerzen. Der Tumor ist stark infiltrativ, oft mit vaskulärer, lymphatischer und perineuraler Invasion verbunden, mit anschließender hämatogener Ausbreitung und frühem Rezidiv."},"es":{"name":"Carcinoma adenoide quístico de cuello de útero","synonyms":["Carcinoma adenoide quístico cervical"],"definition":"Es un cáncer uterino extremadamente agresivo y poco frecuente que aparece macroscópicamente como una masa polipoide irregular no-friable de crecimiento lento en el cuello uterino, y que histológicamente muestra un patrón de crecimiento pseudoglandular o cribiforme. Clinicamente, se manifiesta con sangrado y secreción vaginal y dolor abdominal o pélvico. El tumor es sumamente infiltrativo, asociándose a menudo con invasión vascular, linfática y perineural, con posterior propagación hematógena y recidiva temprana."},"it":{"name":"Carcinoma adenoide cistico della cervice uterina","synonyms":["Carcinoma adenoide cistico cervicale"],"definition":"Il carcinoma adenoide cistico della cervice uterina è un tumore raro, molto aggressivo dell'utero. Dal punto di vista macroscopico si presenta come una massa polipoide irregolare, non friabile, a crescita lenta, localizzata nella cervice uterina. Dal punto di vista istologico, presenta una crescita pseudoghiandolare o cribriforme. Esordisce con emorragie e perdite vaginali e con dolore addominale o pelvico. Il tumore è molto infiltrante e spesso si associa a invasione vascolare, linfatica e perineurale, con conseguente diffusione per via ematica e ricorrenza precoce."},"nl":{"name":"Adenoïd cystisch carcinoom van cervix uteri","synonyms":["Adenoïd cystisch carcinoom van baarmoederhals","Cervicaal adenoïd cystisch carcinoom"],"definition":"Een zeldzame, zeer agressieve kanker van uterus, die macroscopisch voorkomt als een onregelmatige, traaggroeiende, niet-brokkelige, polypoïde massa op de baarmoederhals en die histologisch een pseudoglandulair of cribriform (zeefvormig) groeipatroon vertoont. Het manifesteert zich met vaginale bloeding en afscheiding, en buik- of bekkenpijn. De tumor is zeer infiltratief, vaak geassocieerd met vasculaire, lymfatische en perineurale invasie, gevolgd door hematogene uitzaaiing en vroege terugkeer."},"pt":{"name":"Carcinoma adenóide quístico do colo do útero"},"pl":{"name":"Rak gruczołowato-torbielowaty szyjki macicy","synonyms":["Rak gruczołowato-torbielowaty szyjki"]},"cs":{"name":"Adenoidně cystický karcinom děložního čípku","synonyms":["Cervikální adenoidně cystický karcinom"]},"uk":{"name":"Аденоідно-кістозна карцинома шийки матки","synonyms":["Аденоідно-кістозна карцинома шийки матки"],"definition":"A rare, highly aggressive uterine cancer, macroscopically appearing as an irregular, slow-growing, non-friable, polypoid mass on the uterine cervix and histologically showing a pseudoglandular or cribriform growth pattern. It presents with vaginal bleeding and discharge and abdominal or pelvic pain. The tumor is highly infiltrative, often associated with vascular, lymphatic and perineural invasion, with subsequent haematogenous spread and early recurrence."},"zh":{"name":"子宫颈腺样囊性癌","synonyms":["宫颈腺样囊性癌"],"definition":"一种罕见的高度侵袭性子宫癌，肉眼上表现为子宫颈上不规则、生长缓慢、不易碎的息肉状肿块，组织学上表现为假腺样或筛状生长模式。本病表现为阴道出血和分泌物以及腹部或盆腔疼痛。该肿瘤具有高度浸润性，常有血管、淋巴管和周围神经浸润，随后可致血行扩散和早期复发。"},"tr":{"name":"Serviks uterinin adenoid kistik karsinomu","synonyms":["Servikal adenoid kistik karsinom"]}},"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":"2C77.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"109863869"},{"source":"MONDO","code":"0006133","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1332911","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":213741,"name":"OBSOLETE: Adenoid cystic carcinoma of the corpus uteri","status":"inactive"}],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":213761,"name":"Rare cancer of cervix uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}