Paget's disease of the vulva: Prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision

OBJECTIVE: Our aim was to determine the prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence of Paget's disease of the vulva. STUDY DESIGN: A retrospective review of tumor and pathology registries at 8 institutions is presented. Patients with recurrent disease were excluded. Histologic slide review was performed. RESULTS: The median age of the 100 patients was 70 years. The median duration of pruritus before surgery was 2 years. Thirty-four percent of patients experienced a recurrence at a median of 3 years. There was a 12% prevalence of invasive vulvar Paget's disease and a 4% prevalence of associated vulvar adenocarcinoma. One patient died of Paget's disease with associated vulvar adenocarcinoma. CONCLUSIONS: Paget's disease of the vulva is rarely associated with an underlying vulvar adenocarcinoma or invasive Paget's disease, but there is a high recurrence rate.