Extramammary Paget’s Disease

Abstract

I read with interest the article by Pierie and coauthors concerning extramammary Paget’s disease. I noted their results in 33 cases of a 42% recurrence rate and 52% incidence of positive margins on permanent sections. They claimed that “one would not expect intraoperative frozen section analysis of margins to substantially reduce the incidence of microscopically positive margins on permanent sections.” I would like to point out a study by Stacy and colleagues of Atlanta, GA, that showed that intraoperative frozen sections of margins in patients with extramammary Paget’s disease of the vulva did reduce the need for subsequent operations. They reported on 13 consecutive cases of vulvar Paget’s disease. In a group of eight patients having Paget’s disease not involving the anal mucosa, the extent of disease was completely defined by frozensection margins. Additional intraoperative resections were necessary in five of the eight. None had residual involvement on permanent sections and none had recurrences in 3 to 8 years of followup. A second group of two patients had frozen sections to define some, but not all, of the margins. Both had positive perineal margins on permanent sections. One required two subsequent revisions after 3 years of followup, and the other was free of disease during 9 years of followup. A third group of three patients all had anal mucosal involvement, and all had associated mucinous adenocarcinoma of the rectum. Frozen section actually discovered the presence of one of the three carcinomas. After appropriate radical operations, all three were alive and free of disease between 2 and 9 years of followup. I worked with the Atlanta group from 1976 to 1984. Since 1979, I have used intraoperative frozen sections to evaluate margins and to define the extent of surgery necessary. In my experience, this minimizes recurrences of Paget’s disease of the vulva. I believe that frozen sections for margins at the time of wide local excision is both indicated and mandatory. This lesion always extends beyond its gross margin. Although a 2-cm margin is usually adequate, many times it is not. I believe that the high recurrence rate quoted by Pierie and colleagues is not from the multifocal nature of Paget’s disease, but rather from inadequate surgery and inadequate margins. Between 1984 and 2002, I personally operated on 15 women with Paget’s disease of the vulva. Five had a partial vulvectomy, one had a hemivulvectomy, seven had a simple complete bilateral vulvectomy, and two had an extended radical vulvectomy with pedicle flap reconstruction. In all cases, frozen section evaluation of the margins was used to determine the extent of resection required and to try to ensure that such patients would not require subsequent anesthesia and operations for extramammary Paget’s disease. Disease recurred in 2 of 15 patients (13%). Both had been treated with extended radical vulvectomy because the disease had initially spread beyond the vulva. One recurred on the mons at 8 months, and one at the vaginal margin 16 months later. They remained free of disease after wide local excisions with a followup of 6 years and 1 year, respectively. In my opinion, women with Paget’s disease of the vulva should be referred to a gynecologic oncologist for appropriate surgical management.