The Roswell Park Cancer Institute experience with extramammary Paget's disease

Abstract

Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm. Common sites of occurrence include the vulva, perianal region, perineum and scrotum. Despite frequent recurrences, surgery is the standard treatment. This study examines the recurrence rate for EMPD treated by conventional surgical management. Alternative and multimodal therapeutic approaches are reviewed. This retrospective analysis included all 30 patients treated for EMPD at Roswell Park Cancer Institute (RPCI) between 1970 and 1998. Following conventional surgical treatment, 44% of our patients developed recurrence. Vulvectomy provided the lowest recurrence rate, but involved extensive tissue loss and functional debility. Multimodal treatment using Mohs' micrographic surgery and photodynamic therapy has been used at RPCI to manage EMPD with minimal tissue loss and no functional impairment. Surgical treatment offers a moderate chance of EMPD cure. Long-term multimodal approaches require close follow-up, but may conserve both tissue and function.