Epidemiology and treatment of extramammary Paget disease in the Netherlands

Aim To determine the incidence of EMPD and to describe its epidemiology, treatment, survival and the risk of developing other malignancies. Method All cases of EMPD, diagnosed between 1989 and 2001, were selected from the Netherlands Cancer Registry. Results In total, 178 cases of invasive and 48 cases of in situ EMPD had been registered. The overall relative 5-year survival for invasive tumours was 72%. Most patients with invasive as well as in situ cancer underwent surgery. Other malignancies were found in 32% of patients with invasive EMPD and 35% of patients with in situ EMPD. Patients had an increased risk of developing a second primary cancer (standardized incidence ratio: 1.7; 95% confidence interval 1.2–2.4). The most frequent localizations of the other cancers were the colorectum, the prostate, the breast and the extragenital skin. Conclusions For EMPD, which is a rare disease in the Netherlands, there are no clear diagnostic and treatment guidelines. The prognosis is fairly good. A thorough search for other tumours is recommended for these patients.