The patients with Peutz-Jeghers syndrome have a high risk of developing cancer

Özet

Peutz-Jeghers syndrome is an autosomal dominant disorder characterized by mucocutaneous hyperpigmentation,and intestinal and extraintestinal multiple hamartomatous polyps. Development of gastrointestinal and extragastrointestinalcancer risk is markedly increased in patients with Peutz-Jeghers syndrome. We analyzed five patientsfrom two families diagnosed with Peutz-Jeghers syndrome between 1999 and 2012. This study confirms the actualmalignancy potency of PJS. Therefore, we suggest a close follow-up of patients with Peutz-Jeghers syndrome for therisk of malignancy.