# Vulvar melanoma

Source: https://onco.cc/cancers/vulvar-melanoma/  
OnCo record `vulvar-melanoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Vulvar melanoma is the second most common vulvar cancer, a melanoma of the mucosal skin of the vulva in older women that is usually found late and has a poorer outlook than skin melanoma. It is removed with a margin, staged like skin melanoma by thickness, and treated when advanced with the immunotherapy and, for the quarter with a BRAF or KIT mutation, the targeted drugs used for other melanomas.

## Summary

Primary vulvar melanoma is the second most common vulvar malignancy and is staged by the American Joint Committee on Cancer system for cutaneous melanoma despite its distinct site and genetics; in 100 patients, older age, greater thickness, higher dermal mitotic rate, ulceration, lymphovascular and perineural invasion, microscopic satellitosis and absence of a precursor naevus were associated with worse survival, and thickness and mitotic rate robustly predicted melanoma-specific survival (Clin Cancer Res 2017). A review of 33 patients found the expected profile of older women with delayed presentation, high stage and poor prognosis, lichen sclerosus in 9.1 percent, and c-KIT expression as a prognostic marker (Int J Mol Med 2014). Molecular profiling of 51 vulvar and vaginal melanomas against 2,253 non-gynaecological melanomas found BRAF the most frequently mutated gene (26 percent, against 8.3 percent of other mucosal melanomas), with fewer of the BRAF mutations at the valine 600 codon (Cancer 2017). Incidence differs less by race than cutaneous melanoma, with a white to black ratio of 3.14 to 1 for vulvar melanoma (Melanoma Research 2010).

How it differs from its parent: it is not a carcinoma, so HPV, p16 and the squamous pathways do not apply; staging follows melanoma thickness rather than FIGO; and systemic therapy follows the mucosal melanoma page.

How common: about 1 to 2 per million women a year (Melanoma Research 2010).

Treatment: wide local excision with margins by thickness rather than radical vulvectomy, sentinel node biopsy, and adjuvant or metastatic therapy as on the mucosal melanoma page: checkpoint inhibitors (nivolumab, pembrolizumab, ipilimumab), BRAF and MEK inhibitors for BRAF V600 mutations and imatinib for KIT mutations (Cancer 2017 for the mutation frequencies).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Primary vulvar melanoma; Melanoma of the vulva; Vulvovaginal melanoma (with vaginal melanoma)
- Tags: subtype-page; wave4; rare
- Group: gynaecologic
- Burden: The second most common vulvar malignancy (Clin Cancer Res 2017); 324 vulvar melanomas were diagnosed in the US SEER registries over 1992 to 2005, with an annual incidence of 1.90 per million non-Hispanic white women and 0.87 per million black women (Melanoma Research 2010).
- Subtypes: Vulvar melanoma of the labia and clitoris (mucosal melanoma); Vulvar melanoma arising in lichen sclerosus (9 percent in one series); BRAF-mutated vulvar melanoma (26 percent of vulvovaginal melanomas); KIT-mutated vulvar melanoma
- Biomarkers: Breslow thickness and dermal mitotic rate (survival); Ulceration, lymphovascular and perineural invasion, satellitosis; BRAF, KIT and NRAS mutation testing; Sentinel node status

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/vulvar-melanoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/vulvar-melanoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/vulvar-melanoma/#what-it-is [4 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/vulvar-melanoma/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/vulvar-melanoma/#treating-it [1 setting, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/vulvar-melanoma/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/vulvar-melanoma/#science [4 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/vulvar-melanoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/vulvar-melanoma/#living-with-it [8 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/vulvar-melanoma/coming/ [4 medicines]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/vulvar-melanoma/data/ [14 connected records]

## Standard of care

- All stages: Wide local excision by thickness with sentinel node biopsy; systemic therapy as on the mucosal melanoma page: checkpoint inhibitors, BRAF and MEK inhibitors for BRAF V600, imatinib for KIT. ([Vulvar cancer](https://onco.cc/cancers/vulvar/), [Mucosal melanoma](https://onco.cc/cancers/mucosal-melanoma/), [Nivolumab](https://onco.cc/drugs/nivolumab/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [Ipilimumab](https://onco.cc/drugs/ipilimumab/), [Imatinib](https://onco.cc/drugs/imatinib/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Vulvar_cancer
- NCI PDQ: vulvar cancer treatment: https://www.cancer.gov/types/vulvar/treatment
- Clin Cancer Res 2017: tumour thickness and mitotic rate predict survival in 100 primary vulvar melanomas: https://doi.org/10.1158/1078-0432.ccr-16-2126
- Int J Mol Med 2014: clinicopathological review of 33 vulvar melanomas, c-KIT as a prognostic marker: https://doi.org/10.3892/ijmm.2014.1659
- Cancer 2017: vulvar and vaginal melanoma, molecular analysis of 51 cases against 2,253 non-gynaecological melanomas: https://doi.org/10.1002/cncr.30473
- Melanoma Research 2010: population-based incidence of vulvar and vaginal melanoma by race and ethnicity, SEER 1992 to 2005: https://doi.org/10.1097/cmr.0b013e32833684e8

## Connected records

- cancers: [Bartholin gland carcinoma](https://onco.cc/cancers/bartholin-gland-carcinoma/), [Melanoma](https://onco.cc/cancers/melanoma/), [Melanoma of the urethra](https://onco.cc/cancers/urethral-melanoma/), [Mucosal melanoma](https://onco.cc/cancers/mucosal-melanoma/), [Vaginal melanoma](https://onco.cc/cancers/vaginal-melanoma/), [Vulvar cancer](https://onco.cc/cancers/vulvar/)
- targets: [BRAF](https://onco.cc/targets/braf/), [KIT](https://onco.cc/targets/kit/)
- drugs: [Imatinib](https://onco.cc/drugs/imatinib/), [Ipilimumab](https://onco.cc/drugs/ipilimumab/), [Nivolumab](https://onco.cc/drugs/nivolumab/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/)

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JSON: https://onco.cc/api/v1/entities/vulvar-melanoma.json