Adenoid cystic carcinoma of the cervix uteri
Synonyms: Cervical adenoid cystic carcinoma
- Type
- Disease
- Group
- Disorder
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Adenoid cystic carcinoma of the cervix uteri | Cervical adenoid cystic carcinoma |
| fr | Carcinome adénoïde kystique du col de l'utérus | Carcinome adénoïde kystique cervical |
| de | Adenoides zystisches Karzinom der Cervix uteri | Zervixkarzinom, adenoides zystisches |
| es | Carcinoma adenoide quístico de cuello de útero | Carcinoma adenoide quístico cervical |
| it | Carcinoma adenoide cistico della cervice uterina | Carcinoma adenoide cistico cervicale |
| nl | Adenoïd cystisch carcinoom van cervix uteri | Adenoïd cystisch carcinoom van baarmoederhals · Cervicaal adenoïd cystisch carcinoom |
| pt | Carcinoma adenóide quístico do colo do útero | |
| pl | Rak gruczołowato-torbielowaty szyjki macicy | Rak gruczołowato-torbielowaty szyjki |
| cs | Adenoidně cystický karcinom děložního čípku | Cervikální adenoidně cystický karcinom |
| uk | Аденоідно-кістозна карцинома шийки матки | Аденоідно-кістозна карцинома шийки матки |
| zh | 子宫颈腺样囊性癌 | 宫颈腺样囊性癌 |
| tr | Serviks uterinin adenoid kistik karsinomu | Servikal adenoid kistik karsinom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C53.0 | BTNT | attributed | validated |
| ICD-10 | C53.1 | BTNT | attributed | validated |
| ICD-10 | C53.8 | BTNT | attributed | validated |
| ICD-11 | 2C77.1 | NTBT | index | validated |
| MONDO | 0006133 | E | validated | |
| UMLS | C1332911 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:213761Rare cancer of cervix uteri
rare neoplastic diseases; rare gynecological and obstetric diseases
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:213741OBSOLETE: Adenoid cystic carcinoma of the corpus uteri referred to · inactive
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/213823
- This record as JSON: /data/d/213823.json