[Update regarding the management of vulvar cancer: The guidelines of the Assistance Publique-Hôpitaux de Paris]

Abstract

Vulvar cancer is a rare disease, which represents 4% of gynecological tumors with an incidence of 0.5 to 1.5 per 100,000 women per year in France. Vulvar cancers are induced in 30 to 69% of cases by the presence of papillomavirus (HPV), in particular HPV 16 and 18, and can also occur in an inflammatory context. The diagnosis is made by histological examination of a vulvar biopsy. The histological subtype is a squamous cell carcinoma in 90% of cases. The 5-year survival of patients with vulvar cancer ranges from 86% for localized stages (FIGO I and II) to 57% for advanced stages (FIGO III and IVA), and 17% in case of metastatic disease (FIGO IVB). The treatment of vulvar cancer is mainly surgical, but radiotherapy and chemotherapy have become more important in recent years. Management has evolved into a personalized multidisciplinary approach, where each therapeutic decision must be discussed in a multidisciplinary consultation meeting. Surgical excision with tumor- free margins is central in the management of early stages. The indication for radiotherapy and brachytherapy should be discussed in the event that the excisional margins are positive in early stages. Radiotherapy is indicated in cases of lymph node involvement or in a neoadjuvant situation if the tumor is not immediately resectable. In this situation, it can be associated with chemotherapy. Chemotherapy alone is the treatment of diseases that are metastatic at the time of diagnosis.

Keywords: Cancer de la vulve; Cancers gynécologiques; Gynecological cancers; Papillomavirus; Vulvar carcinoma.