Long-term outcome of patients with perianal Paget's disease.

Abstract

Perianal Paget's disease (PPD) is a rare intraepithelial adenocarcinoma with a significant rate of recurrence after treatment and high risk of progression to an invasive cancer. Fourteen patients with a mean follow-up longer than 5 years were studied to determine the outcome after surgical treatment. The immunohistochemical accumulation of p53 protein also was assessed in tissue specimens to evaluate its prognostic role in patients with PPD. Four patients were excluded because of progression to invasive malignancy at the time of diagnosis. Two patients underwent local excision (LE) with macroscopic clearance of the surgical margins; the remaining eight patients underwent wide local excision (WLE), i.e., > 1 cm microscopic clearance of the surgical margins. The actuarial 8-year recurrence rate for patients treated with LE and WLE was 100% and 50% (SE = 17.7), respectively. Progression to invasive carcinoma occurred after a median time of 56 months (range 23-72) in two patients treated with LE and in one of eight patients treated with WLE. All four patients with recurrence after WLE were successfully treated (no further recurrence) with a second WLE. Actuarial 8-year survival was 0% in the LE group and 40% (SE = 21.9) in the WLE group. There was no p53 protein accumulation in any of the ten patients with PPD. Survival of patients with PPD treated by WLE was higher than that for those treated with LE. Thus, wide local excision is recommended over limited local excision as a preferred treatment for PPD. Follow-up longer than 5 years seems to be indicated because of the risk of late progression to invasive cancer. When PPD does recur, a second WLE may be curative. The absence of accumulated p53 protein suggests that this marker may not have a prognostic role in PPD.