Total vaginectomy and radical vulvectomy for extension of extra-mammary Paget's disease.

Extra-mammary Paget's disease of the vulva is a rare non-invasive adenocarcinoma that usually occurs in postmenopausal women. Histologically, it often extends beyond the visible lesion, leading to positive surgical margins and frequent recurrences, but can be managed by simple vulvectomy or wide local excision. Although current evidence supports the use of radical surgery as an alternative to the generally performed wide local excision in the treatment of widely extended extra mammary Paget's disease of the vulva, nonetheless there controversy still exists regarding the extent of an adequate resection margin. Here the authors present a case of successful radical vulvectomy with total vaginectomy without adjuvant treatment on a delayed diagnosis of extra-mammary Paget's disease, extending from the vulva to the apex of vagina.