Invasive cancer of the vulva

Invasive vulvar cancer is a relatively rare tumor, accounting for 4% of all female genital malignant neoplasms. Although classically a disease of elderly women, the trend in recent years is an increasing prevalence among younger women, which cannot be accounted for by immune suppression alone. Human papillomavirus (HPV) is a key age-dependent risk factor that causes preinvasive disease in the form of vulvar intraepithelial neoplasia (VIN) that is often associated with a history of tobacco use. HPV-related VIN lesions are rarer in older women, and these malignancies may be associated with chronic vulvar dystrophies, such as lichen sclerosis, although a direct association remains unproven. Staging is determined surgically and impacts prognosis significantly, with early stages having favorable prognoses while advanced stages portend a much poorer prognosis. Early-stage cancers are managed surgically in most cases with radical excision and lymphadenectomy. Advances in techniques have favored decreasing radicality of surgery with closer margins and sentinel lymph node biopsy. Locally advanced tumors often require combined chemoradiation. Metastatic disease is treated with systemic therapies, traditionally with cytotoxic chemotherapy, though immunotherapy is gaining favorability as more encouraging data are resulting. Recurrences may be local or distant, and more than 80% will occur in the first 2 years after therapy, demanding initial close follow-up.