A case of Peutz-Jeghers syndrome with nasal polyposis, extreme iron deficiency anemia, and hamartoma-adenoma transformation: management by combined surgical and endoscopic approach.

Abstract

We report a rare variant of Peutz-Jeghers syndrome identified by the presence of nasal polyposis and extreme anemia. Multiple hamartomatous polyps were found throughout the upper and lower gastrointestinal tract. We conducted a combined surgical-endoscopic approach to prevent the development of a short-bowel syndrome. The polyps removed by snare and surgical polypectomy showed features typical of harmartomas. Three colonic polyps and two nasal polyps showed histological evidence of adenomatous change. This adenomatous change seems to fit into the pathogenic sequence of hamartoma-adenoma.