Vulvar intraepithelial neoplasia
Synonyms: VIN · Vulvar intraepithelial tumor
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare vulvovaginal tumor characterized by intraepithelial neoplastic proliferation of the vulvar epithelium, histologically presenting proliferation of atypical basal cells with basal layer involvement, enlarged nuclei, hyperchromasia, pleomorphic cells and increased numbers of mitotic figures. Patients are frequently asymptomatic, although vulvar pruritus/pain/burning, dysuria and/or dyspareunia may be associated. Concurrent anogenital involvement is frequent. Two subtypes, usual type VIN (uVIN) and differentiated type VIN (dVIN) exist, with uVIN typically being associated with HPV infection and presenting multifocal, elevated lesions around the introitus and/or labia majora, and dVIN being related to chronic inflammation and lesions consisting of poorly demarcated pink or white plaques that are often associated with lichen sclerosus or lichen planus. Diffusely positive p16 immunohistochemistry and high Ki-67 proliferation index in uVIN futher differentiates this subtype from dVIN, this latter being consistently negative for p16 while presenting p53 positivity.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Vulvar intraepithelial neoplasia | VIN · Vulvar intraepithelial tumor |
| fr | Néoplasie intraépithéliale de la vulve | Tumeur intraépithéliale de la vulve · VIN |
| de | Vulväre intraepitheliale Neoplasie | VIN · Vulvärer intraepithelialer Tumor |
| es | Neoplasia vulvar intraepitelial | NVI · Tumor vulvar intraepitelial |
| it | Neoplasia vulvare intraepiteliale | Tumore vulvare intraepiteliale · VIN |
| nl | Vulvaire intra-epitheliale neoplasie | Intra-epitheliale neoplasie van vulva · VIN · Vulvaire intra-epitheliale tumor |
| pt | Neoplasia vulvar intraepitelial | Tumor intraepitelial vulvar · VIN |
| pl | Nowotwór wewnątrznabłonkowy sromu | Guz śródnabłonkowy sromu · Śródnabłonkowa neoplazja sromu |
| cs | Vulvární intraepiteliální neoplázie | Vulvární intraepiteliální nádor |
| uk | Вульварна інтраепітеліальна неоплазія | VIN · Вульварна інтраепітеліальна пухлина |
| zh | 外阴上皮内瘤变 | 外阴上皮内肿瘤 |
| tr | Vulvar intraepitelyal neoplazi | VIN · Vulvar intraepitelyal tümör |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D07.1 | NTBT | inclusion | validated |
| ICD-11 | 2E67.12 | BTNT | specific | validated |
| ICD-11 | 2E67.13 | BTNT | specific | validated |
| ICD-11 | GA13.1 | BTNT | specific | validated |
| MONDO | 0005198 | E | validated | |
| UMLS | C0346210 | 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:180312Rare vulvovaginal tumor
rare neoplastic diseases; rare gynecological and obstetric diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 20 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2008[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/137583
- This record as JSON: /data/d/137583.json