PEUTZ-JEGHERS SYNDROME WITH MALIGNIZATION OF A HAMARTOMATOUS POLYP

Peutz-Jeghers syndrome is characterized by hamartomatous polyps in the gastrointestinal tract associated with mucocutaneous melanin spots; the condition is inherited in an autosomic dominant fashion. Although the hamartomatous polyps are not regarded as malignant, there is an association between the Peutz-Jeghers syndrome and the development of carcinomas of the gastrointestinal tract. Similary, there is an increasing evidence suggesting that hamartomas can give rise to adenocarcinomas. We present a patient with Peutz-Jeghers syndrome who developed an adenocarcinoma in an hamartomatous jejunal polyp.