VULVAR PAGETS-DISEASE - 36 CASES

Thirty six patients with Paget's disease of the vulva were reviewed. T he median age of the patients at diagnosis was 67 years (range : 45-91 years). One patient had a history of previous mammary adenocarcinoma. Screening for malignancy revealed two colonic tumours. Two patients w ith negative screening at presentation developed, 12 and 18 months res pectively after vulvectomy, an ovarian carcinoma stage IIc and a cervi cal and urethral adenocarcinoma. All patients were treated by surgery based on extent of the disease. The operations performed included tota l vulvectomy (n = 11), partial vulvectomy (n = 14) and wide local exci sion (n = 4). Out of the 36 patients, 29 were available to follow-up. The median follow-up period was 74 months (range 2-204 months). Three patients died of metastatic disease due to vulval adenocarcinoma and b reast carcinoma, or of liver metastases from an unknown adenocarcinoma . Eighteen of the 29 patients followed up remained free of disease. Fi ve out of the 16 patients with positive margins recurred, as did 5 out of 9 patients with negative margins. Treatment of Paget's disease of the vulva is surgical. In order to prevent recurrence, some authors ha ve proposed surgical excision extending beyond the visible clinical le sions with intraoperative frozen sections. The data we recorded show t hat free margins do not seem to correlate with recurrence, so that lar ge excision beyond the clinical lesion is not useful.