ICD-11 code : 2E67.11;
Preferred Label : Vulvar Paget disease;
ICD-11 definition : An uncommon intraepithelial malignant neoplasm of eccrine or apocrine origin, arising
from the vulva. It usually affects post-menopausal women. In approximately 10-20%
of the cases there is an associated anorectal, or urothelial carcinoma or a skin appendage
adenocarcinoma identified. It presents as a red, eczematous lesion. Microscopically,
it is characterized by the presence of the typical Paget cells which are large, round
cells with abundant cytoplasm and prominent nuclei.;
ICD-11 synonym : Extramammary Paget disease of vulva;
Origin ID : 1913185137;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- ICD-10 Mapping
An uncommon intraepithelial malignant neoplasm of eccrine or apocrine origin, arising
from the vulva. It usually affects post-menopausal women. In approximately 10-20%
of the cases there is an associated anorectal, or urothelial carcinoma or a skin appendage
adenocarcinoma identified. It presents as a red, eczematous lesion. Microscopically,
it is characterized by the presence of the typical Paget cells which are large, round
cells with abundant cytoplasm and prominent nuclei.
