Vulvar malignancies are rare with squamous cell carcinoma (SCC) as the most common histological type. Vulvar basal cell carcinomas and melanomas are even more rare. In general, vulvar cancer is a disease of the elderly woman but the incidence is increasing, especially in younger women. There are two oncogenic pathways of vulvar SCCs, both with their own premalignancy, but with comparable treatment until now. Surgery is the cornerstone of treatment consisting of excision of the primary tumour with groin surgery. This leads to a 5-year survival rate of around 70 %. The treatment of vulvar cancer has an enormous impact on the psychosexual quality of life. Lichen sclerosus, vulvar high-grade squamous intra-epithelial lesion (HSIL), differentiated vulvar intraepithelial neoplasia (dVIN) and Paget’s disease of the vulva are all vulvar premalignancies with a broad spectrum of symptoms. Dedicated multidisciplinary teams are necessary to optimize cure and care for women with vulvar cancer and vulvar premalignancies.
