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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.
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).
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).
Most high-grade ovarian cancers begin at the tip of the fallopian tube; endometrial cancer lines the uterus, cervical cancer starts at the transformation zone; each drains to a different node group.
Same organ: Adenosquamous carcinoma of the cervix, Small cell neuroendocrine carcinoma of the cervix, Bartholin gland carcinoma, Vaginal melanoma, High-grade serous ovarian cancer, Low-grade serous ovarian cancer, Clear cell ovarian cancer, Mucinous ovarian cancer, Adult granulosa cell tumour of the ovary, Ovarian cancer, Endometrial cancer, Cervical cancer, Vulvar cancer, Gestational trophoblastic neoplasia, Uterine sarcoma, Vaginal cancer, POLE-ultramutated endometrial cancer, Mismatch-repair-deficient endometrial cancer, p53-abnormal endometrial cancer, including uterine serous carcinoma, Endometrial cancer with no specific molecular profile, Advanced or recurrent endometrial cancer, Uterine carcinosarcoma, Early cervical cancer and fertility-sparing surgery, Locally advanced cervical cancer, Recurrent or metastatic cervical cancer, Platinum-sensitive ovarian cancer, Platinum-resistant ovarian cancer, HPV-associated vulvar squamous cell carcinoma, HPV-independent vulvar squamous cell carcinoma (p53-mutant), Vaginal squamous cell carcinoma (HPV-associated), Vaginal adenocarcinoma (including DES-associated clear cell adenocarcinoma), Low-risk gestational trophoblastic neoplasia (FIGO score 0 to 6), High-risk gestational trophoblastic neoplasia (FIGO score 7 or more, including ultra-high-risk), Placental-site trophoblastic tumour and epithelioid trophoblastic tumour
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.
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Query for this cancer: (TITLE:"Vulvar melanoma" OR ABSTRACT:"Vulvar melanoma" OR TITLE:"Primary vulvar melanoma" OR ABSTRACT:"Primary vulvar melanoma" OR TITLE:"Melanoma of the vulva" OR ABSTRACT:"Melanoma of the vulva" OR TITLE:"Vulvovaginal melanoma with vaginal melanoma" OR ABSTRACT:"Vulvovaginal melanoma with vaginal melanoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Vulvar melanoma, not a curated reading list.
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Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Take with a meal and a large glass of water.
No pharmacokinetic interactions expected (antibody). See the irAE guide for toxicity management.
Immunotherapy can attack hormone-producing glands: most often the thyroid (usually ending in an under-active thyroid needing lifelong tablets), and less often the pituitary (hypophysitis) or adrenal glands, which can be life-threatening if missed.
Inflammation of the bowel caused by immunotherapy releasing the immune system against the gut lining, producing diarrhoea that can be severe. It is the commonest serious side effect of CTLA-4 antibodies and is treated with steroids and, if needed, infliximab.
See all on the product pages:ImatinibIpilimumabNivolumabPembrolizumab·Printable cards in the navigator
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