Vulvar melanoma is the second most common vulvar cancer.,Patients with vulvar melanoma usually present with the disease at a late stage and have a poor prognosis.,The prognostic predictors reported in the literature are not unequivocal and the role of lichen sclerosus and c-KIT mutations in the aetiology of vulvar melanoma is unclear.,Breslow staging currently seems to be the most adequate predictor of prognosis.,We thus performed a clinicopathological and literature review to identify suitable predictors of prognosis and survival and investigated the expression of c-KIT (by immunohistochemistry) in patients with vulvar melanoma (n=33) from the Gynaecological Cancer Centres of the Royal Hospital for Women (Sydney, Australia) and John Hunter Hospital (Newcastle, Australia).,Our series of 33 patients fitted the expected clinical profile of older women: delayed presentation, high stage, limited response to treatment and poor prognosis.,We identified 3 patients (9.1%) with lichen sclerosus associated with melanoma in situ, although no lichen sclerosus was found in the areas of invasive melanoma.,No patient had vulvar nevi.,We identified a) Breslow’s depth, b) an absence of any of the pathological risk factors, such as satellitosis, in-transit metastasis, lymphovascular space invasion (LVSI) and dermal mitosis, c) removal of inguino-femoral lymph nodes, d) lateral margin of >1 cm, and e) c-KIT expression as valuable prognostic predictors for disease-free survival.,We conclude that c-KIT expression is, apart from Breslow’s depth, another valuable predictor of prognosis and survival.,Lichen sclerosus may be associated with vulvar melanoma.
Mutations in KIT are more frequent in specific melanoma subtypes, and response to KIT inhibition is likely to depend on the identified mutation.,A total of 32 patients with metastatic acral or mucosal melanoma were screened for mutations in KIT exons 11, 13 and 17.,KIT mutations were found in 38% of mucosal and in 6% of acral melanomas.,Three patients were treated with imatinib and one with sorafenib.,All four patients responded to treatment, but three have since progressed within the brain.,The observed clinical responses support further investigation of KIT inhibitors in metastatic melanoma, selected according to KIT mutation status.