Paget's disease of the vulva.

Thirty-six patients with Paget's disease of the vulva were reviewed. Median age of the patients at diagnosis was 64 years (range 41-84 years). Five patients (14%) had an associated invasive adenocarcinoma of the vulva at the time of diagnosis. Of 31 patients with superficial noninvasive Paget's disease, 28 were available for follow-up. Treatment of patients with superficial Paget's disease was surgical and based on the extent of disease. Procedures performed included total vulvectomy (25), wide local excision (4), and skinning vulvectomy with skin graft (1). The median follow-up was 108 months (range 6-266 months). Twenty-two of twenty-eight patients remained free of disease. Six patients have required multiple procedures for recurrent superficial Paget's disease. Treatment of Paget's disease of the vulva is surgical. Radical surgery is the preferred treatment of patients with an associated invasive adenocarcinoma; three of five patients with an invasive adenocarcinoma were long-term survivors. Patients with superficial Paget's disease of the vulva should be treated by local excision utilizing frozen-section margin evaluation as a guide to extent of excision. Only one patient with an initial diagnosis of superficial Paget's disease of the vulva developed invasive adenocarcinoma.