Paget's Disease of the Vulva: Pathology, Pattern of Involvement, and Prognosis

Abstract

Gynecol Oncol 2000;77:183–89 This is a retrospective review of 76 patients with Paget's disease of the vulva from the MD Anderson Cancer Center. The authors also divided patients into four groups by diagnosis, the majority (46) being intraepithelial Paget's disease. Nine patients had invasive Paget's disease; an additional 13 patients had intraepithelial Paget's disease with another coexisting cancer. Coexisting cancers of interest included endometrial, ovarian, breast, and cervical carcinomas. The mean age of patients at diagnosis was 67.5 years. Surgical treatment included wide local excision, simple vulvectomy, and radical vulvectomy equal in numbers. Wide local excision appeared to be as effective overall in treating the condition as other modalities. Wide local excision was associated with a higher risk of recurrent disease, but, overall, patients with wide local excision tended to survive longer than patients treated more radically. Comment: This paper is an important study and follow-up of this unusual disease of the vulva. It is written well. It highlights that most Paget's disease of the vulva is intra-epithelial. Involved margins are quite common and do not necessarily correlate to disease recurrence. Additionally, it highlights the problem of underlying adenocarcinoma in a number of patients and the possibility of coexisting cancers. Within this study, there were coexisting cancers of the endometrium, ovary, breast, and cervix. Paget's disease also has been described in other studies with bladder carcinoma and colon cancer. Concerning margin status, the authors point out that margins were positive in 53% of patients; Paget's disease recurred in 31% of patients with positive margins and 33% of patients with negative margins. It has been my practice not to be concerning with margin status and to observe patients with positive margins. Wide local excision has been my treatment of choice to rule out underlying adenocarcinoma followed by close follow-up of the patients. I believe the readers of the Journal of Lower Genital Tract Disease would benefit from reading this entire article. (CJD)