Paget's disease of the perianal region--an aggressive disease?

Abstract

Perianal Paget's disease is a rare entity, often associated with internal malignancies and a poor prognosis. A chart review of patients with perianal Paget's disease who presented consecutively to Mayo during 25 years (starting in January 1970) was made. Patients included had Paget's disease located in or around the anus (3 cm). Patients were excluded for evidence of spread of vulvaperineal lesions or pagetoid extension of a rectal adenocarcinoma. Histology slides were reviewed, and immunohistochemistry was applied to confirm diagnoses. Follow-up was updated in all patients. Recurrence and survival curves were generated by the Kaplan-Meier method. Survival was compared with an age-matched population by the log-rank test. Thirteen patients, eight females, were diagnosed (age +/- standard deviation of 68.3 +/- 10.6 years). All histologic diagnoses were confirmed with immunohistochemical staining results. Mean follow-up was 6.7 years, 8.8 for living patients. One patient had associated extramammary Paget's disease (scrotum). Lesions were located randomly at the dentate line, anal verge, and/or perianal area. Four patients had associated carcinomas; none of them were visceral. Eleven patients underwent local resection, without adjuvant therapy. Almost all recurrences were treated by wider local excision. The five-year recurrence rate was 61 percent. Overall five-year and ten-year survival was 67 percent, no different from the age-matched population (P = 0.546). These results do not reflect an aggressive nature of perianal Paget's disease, despite a high rate of local recurrence. Both primary lesions and recurrences are susceptible to treatment by wider local resection. Long-term survival is no different from that of the normal age-matched population.