Perianal Paget's disease

Abstract

Seven cases of patients with Paget's disease of the anus are presented. The clinical picture is variable. Treatment and prognosis depend on the presence of an underlying invasive carcinoma. If present, treatment is directed toward the underlying carcinoma. If invasive carcinoma is not present, local excision with clear margins is sufficient. Patients who had Paget's disease of the anus without underlying invasive malignancy were cured, although multiple excisions were required. Patients who had perianal Paget's with invasive carcinoma that had already metastasized to the inguinal nodes did poorly, and all died despite treatment. When extramammary Paget's disease was associated with early localized invasive carcinoma the patients did well. The case for “cancer proneness” in extramammary Paget's disease is supported by this study. Seventy-one per cent of patients who had perianal Paget's disease had associated malignancies, and one patient had three separate carcinomas.