{"orpha":213828,"name":"Adenoid basal carcinoma of the cervix uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Cervical adenoid basal carcinoma"],"definition":"A rare, slow-growing uterine cancer characterized, histologically, by small, well differentiated nests of basaloid cells resembling basal cell carcinoma of the skin, commonly associated with squamous cell carcinoma or squamous intraepithelial lesions. Patients are usually asymptomatic or present with dysfunctional vaginal bleeding, often with no observable lesion on the cervix. Infection with high-risk HPV-types (16 and 33) has been reported in some cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213828/","orphanet_url":"https://www.orpha.net/en/disease/detail/213828","names":{"fr":{"name":"Carcinome adénoïde basal du col de l'utérus","synonyms":["Carcinome adénoïde basal cervical"],"definition":"Un cancer utérin rare, lentement progressif, caractérisé sur le plan histologique par de petits nids bien différenciés de cellules basaloïdes ressemblant à un carcinome cutané basocellulaire, associé fréquemment à un carcinome épidermoïde ou à des lésions squameuses intraépithéliales. Les patientes sont généralement asymptomatiques, ou présentent un saignement vaginal dysfonctionnel, souvent en l'absence de lésions utérines observables. Une infection par les types à haut risque du papillomavirus (16 et 33) a été rapportée dans certains cas."},"de":{"name":"Adenoides Basalzellkarzinom der Cervix uteri","synonyms":["Basalkarzinom, adenoides zervikales"],"definition":"Ein seltener, langsam wachsender Gebärmutterkrebs, der histologisch durch kleine, gut differenzierte Nester basaloider Zellen gekennzeichnet ist, die dem Basalzellkarzinom der Haut ähneln und häufig mit Plattenepithelkarzinomen oder Plattenepithelkarzinomen vergesellschaftet sind. Die Patientinnen sind in der Regel asymptomatisch oder präsentieren sich mit dysfunktionalen vaginalen Blutungen, oft ohne erkennbare Läsion an der Zervix. Eine Infektion mit Hochrisiko-HPV-Typen (16 und 33) wurde in einigen Fällen berichtet."},"es":{"name":"Carcinoma adenoide basal de cuello de útero","synonyms":["Carcinoma adenoide basal cervical"],"definition":"Es un cáncer uterino poco frecuente de crecimiento lento caracterizado, histológicamente, por nidos pequeños y bien diferenciados de células basaloides semejantes al carcinoma de células basales de la piel, comúnmente asociado con carcinoma de células escamosas o lesiones intraepiteliales escamosas. por lo general, las afectadas generalmente están asintomáticas o presentan sangrado vaginal disfuncional, a menudo sin lesiones observables en el cuello uterino. Se ha descrito en algunos casos infección por tipos de VPH de alto riesgo (16 y 33)."},"it":{"name":"Carcinoma adenoide basale della cervice uterina","synonyms":["Carcinoma adenoide basale cervicale"],"definition":"Il carcinoma adenoideo basale della cervice uterina è un tumore raro dell'utero a crescita lenta. Dal punto di vista istologico, è caratterizzato da piccoli nidi ben differenziati di cellule basali che ricordano il carcinoma basocellulare della cute, di solito associati al carcinoma a cellule squamose o a lesioni squamose intraepiteliali. Di solito le pazienti sono asintomatiche, oppure presentano emorragie vaginali disfunzionali, spesso in assenza di evidenti lesioni della cervice. In alcuni casi, sono state descritte infezioni da HPV di tipo ad alto rischio (16 e 33)."},"nl":{"name":"Adenoïd basaalcelcarcinoom van baarmoederhals","synonyms":["Adenoïd basaalcelcarcinoom van cervix uteri","Cervicaal adenoïd basaalcelcarcinoom"],"definition":"Een zeldzame, traaggroeiende kanker van uterus, histologisch gekarakteriseerd door kleine, goed gedifferentieerde nesten van basaloïde cellen die lijken op basaalcelcarcinoom van huid, doorgaans geassocieerd met plaveiselcelcarcinoom of squameuze intra-epitheliale laesies. Patiënten zijn meestal asymptomatisch of vertonen disfunctionele vaginale bloeding, vaak zonder zichtbare laesie aan de baarmoederhals. In enkele gevallen werd infectie met risicovolle HPV-types (16 en 33) gerapporteerd."},"pt":{"name":"Carcinoma adenóide basal do colo do útero"},"pl":{"name":"Rak gruczołowato-podstawnokomórkowy szyjki macicy","synonyms":["Rak gruczołowato-podstawnokomórkowy szyjki"]},"cs":{"name":"Adenoidně bazální karcinom hrdla děložního","synonyms":["Cervikální adenoidně bazální karcinom"]},"uk":{"name":"Аденоїдна базальна карцинома шийки матки","synonyms":["Базальна карцинома аденоїдів шийки матки"],"definition":"A rare, slow-growing uterine cancer characterized, histologically, by small, well differentiated nests of basaloid cells resembling basal cell carcinoma of the skin, commonly associated with squamous cell carcinoma or squamous intraepithelial lesions. Patients are usually asymptomatic or present with dysfunctional vaginal bleeding, often with no observable lesion on the cervix. Infection with high-risk HPV-types (16 and 33) has been reported in some cases."},"zh":{"name":"子宫颈腺样基底癌","synonyms":["宫颈腺样基底癌"],"definition":"一种罕见的、生长缓慢的子宫癌，组织学上为小而分化良好的、类似皮肤基底细胞癌的基底样细胞巢，通常与鳞状细胞癌或鳞状上皮内病变有关。患者通常无症状或出现功能失调性阴道出血，宫颈常无明显病变。在一些病例中曾报道与高危型HPV感染（16型和33型）相关。"},"tr":{"name":"Serviks uterinin adenoid bazal karsinomu","synonyms":["Servikal adenoid bazal 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":"255284408"},{"source":"MONDO","code":"0016287","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"16287","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"6132","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1516403","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"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}