Peutz-jeghers syndrome and colon cancer in a 10-year-old girl: implications for when and how to start screening?

The Peutz-Jeghers syndrome (PJS) is characterized by hamartomatous polyposis of the gastro-intestinal (GI) tract, with mucocutaneous pigmentation. We have experienced a case of a 10-year-old girl who presented with PJS, intussusception, colonic perforation and colonic adenocarcinoma. Finally, this case developed airway obstruction from the mediastinal mass. In order to prevent cancer and short bowel syndrome, aggressive screening is recommended.