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

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