Vulval Paget disease
Vulval Paget disease
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.
Also known as mammalian vulva Paget disease, Paget disease of the vulva, Paget's disease of the vulva, Paget's disease of vulva, vulva Paget's disease, vulval Paget's disease, vulvar Paget's disease — per MONDO
Also identified as
- DOID 2097 per MONDO
- ICD9 233.39 per MONDO
- NCIT C4027 per MONDO
- SCTID 254898001 per MONDO
- UMLS C1275217 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Exocrine system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Female reproductive system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Female reproductive system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Genitourinary system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Genitourinary system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Integumental system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Reproductive system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Skin of body | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Skin of body | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Sweat glands | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Vulva | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Vulva | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |