Paget's disease of the vulva

Our goal was to review our experience with Paget's disease of the vulva relative to initial examination, treatment, and oncologic outcome.

Patients who were treated for extramammary Paget's disease of the vulva at the University of South Florida were identified in our vulvar cancer database for the period 1988 through 2000. The charts were reviewed, and the data were collected regarding patient demographics, previous Paget's treatment, symptoms, surgical margin status, associated malignancies, and time to recurrence.

Twenty-three women with extramammary Paget's disease of the vulva were treated by the Division of Gynecologic Oncology during the 12-year period. The average patient was postmenopausal and white and had symptoms for 21 months before the diagnosis was made. A pruritic lesion was the most common symptom. Treatment included wide local excision or vulvectomy, depending on the extent of disease. Six of the 23 patients displayed invasive disease and, consequently, underwent radical resection. There were 8 recurrences that were found, on average, 30 months after the surgical procedure. Two of the 8 patients had invasive disease at their primary operation, and 1 patient had underlying invasive disease at the time of recurrence. The average follow-up time was 39 months (median, 13.5 months; range, 1-216 months).

Delay in diagnosis did not correlate with size or extent of disease. Margin status did not change the natural course of disease. Recurrence is relatively common, and long-term monitoring is recommended, with repeat excision of symptomatic lesions.