A clinicopathologic study of 30 patients with Paget's disease of the vulva

Clinical and pathologic analyses were performed in 30 patients with Paget's disease of the vulva to investigate the risk factors for recurrence. From 1971 to 1993, we carried out histologic examinations in 27 resected vulvae to detect Paget's cells at the surgical margin. Prior to 1981, 9 patients underwent the examination, and 6 of them had positive surgical margins, and 2 of these 6 patients had local recurrence. After 1982, the preoperative multiple biopsy and intraoperative frozen section diagnosis of the surgical margins were performed, and as a result, cases of the lesions remaining as stumps on the vaginal side decreased in 7 of 18 examined patients. In these 7 cases, 2 cases had local recurrence. The patients were further divided into three groups according to histologic findings to investigate their prognosis: 10 patients with intraepithelial Paget's disease (IEP), 9 patients with minimally invasive Paget's disease (MIP), and 11 patients with underlying carcinoma (UC) including 2 cases of carcinoma in situ. Four locally recurrent cases were found in IEP or MIP, and the longest period from surgery to recurrence was 8 years and 4 months. The invasive UC lesions persisted or recurred shortly after surgery (median, 10 months) and 5 of the 6 patients with general metastasis. For IEP and MIP, surgical margins of the vaginal side should be carefully examined with preoperative multiple biopsy and intraoperative frozen section, and also a long-term follow-up is necessary. With regard to invasive UC patients, their prognosis was found to be very poor for general metastasis.