Testicular tumors with Peutz-Jeghers syndrome

The case of a 6-year-old boy with Peutz-Jeghers syndrome (PJS), gynecomastia, and multifocal and bilateral testicular tumors is described. Females with PJS are known to be at increased risk for developing gonadal tumors. This case and other reports from the literature suggest that males, as well as females, with PJS are at risk for developing gonadal tumors.