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