Management of Advanced Squamous Cell Carcinoma of the Vulva

SIMPLE SUMMARY: Vulvar cancer is a rare gynaecological malignancy that has an increasing incidence, particularly in younger women. Early vulvar cancer can be treated and cured with surgical excision. Approximately 30% of women present with advanced disease, which requires treatment either with mutilating surgery or a combination of chemotherapy and radiotherapy, which is an effective treatment but has many side effects. Current research is focused on new less morbid approaches to treatment, in which drugs that target various steps on the biological pathway from pre-cancer to cancer are used, with the aim of preventing the growth of vulvar cancers. This review is an update of the current management of women with advanced vulvar cancer. ABSTRACT: Vulvar cancer is a rare gynaecological malignancy, accounting for 2–5% of cancers of the female genital tract. Squamous cell carcinoma is the most frequently occurring subtype and, historically, has been a disease of older post-menopausal women, occurring with a background of lichen sclerosus and other epithelial conditions of the vulvar skin that may be associated with well-differentiated vulvar intra-epithelial neoplasia (dVIN). An increase in human papillomavirus (HPV) infections worldwide has led to an increase in vulvar squamous carcinomas in younger women, resulting from HPV-associated high-grade vulvar squamous intra-epithelial lesions (vHSIL). Surgical resection is the gold standard for the treatment of vulvar cancer. However, as approximately 30% of patients present with locally advanced disease, which is either irresectable or will require radical surgical resection, possibly with a stoma, there has been a need to investigate alternative forms of treatment such as chemoradiation and targeted therapies, which may minimise the psychosexual morbidity of radical surgery. This review aims to provide an update on management strategies for women with advanced vulvar cancer. It is hoped that investigation of the molecular biologies of the two different pathways to vulvar squamous cell carcinoma (HPV-associated and non-HPV-associated) will lead to the development of targeted therapeutic agents.