Conservative management of extramammary Paget's disease of the vulva

Patients with extramammary Paget's disease of the vulva at the University of California--Irvine Medical Center and the Memorial Medical Center of Long Beach Women's Hospital between 1976 and 1986 were treated with nonradical surgery. These 14 patients were analyzed in a retrospective fashion to determine if conservative management was successful in eradicating the disease process while preserving appearance and sexual function. All patients were treated with nonradical surgery: skinning vulvectomy with split-thickness skin graft (86%), hemivulvectomy (7%), and simple vulvectomy (7%). In all cases intraoperative frozen-section analysis of surgical margins was performed to determine the extent of disease and outline the margins of resection. No patients had Paget's disease associated with an underlying adenocarcinoma. Three patients developed recurrent Paget's disease. Two of these patients recurred at the site of a positive margin and thus represent persistent disease. Only one patient (7%) had recurrent disease in the face of negative margins. The median duration of follow-up was 50 months. All patients are presently free of disease. The rationale for this conservative management is discussed.