Extramammary Paget's disease with diffuse involvement of the lower female genito-urinary system

Extramammary Paget's disease of the lower female genito-urinary system is an uncommon neoplasm with a high rate of recurrence. A 52-year-old white female with a history of extramammary Paget's disease, originally excised in 1985 by skinning vulvectomy and who had multiple recurrences, including two in a vaginal graft and with extension to the urethra and the uterine cervix, in 1992 underwent an anterior pelvic exenteration for control of the disease process. Standard surgical management for extramammary Paget's disease without invasion or without an underlying adenocarcinoma is simple or skinning vulvectomy. However, because the disease commonly recurs, diffuse involvement may require more extensive surgery including pelvic exenteration in extraordinary cases.