A Case Report of Peutz - Jeghers Syndrome with an Adenomatous Malignant Change in a 44 Year Old Male Patient / 대한소화기내시경학회지

ABSTRACT

Peutz-Jeghers Syndrome, characterized by autosomal inheritance, skin pigmentation, and hamartomatous polyps, had long been considered a benign polyposis until recent studies suggested an association with increased risk of malignant neoplasia in patients who had been followed-up for long periods. The authors present a case of Peutz-Jeghers syndrome in a 44 year old man with an adenomatous malignant change in his small bowel. The patient underwent surgery following a diagnosis of intestinal intussuception. Observing the polyps resected, all the polyps had characteristics of hamartoma, excluding one which showed a malignant transformation. Although it has not been fully determined with certainty whether the adenocarcinoma developed from hamartoma, histologic examination of an excised specimen suggested, thht potency of hamartoma to undergo malignant transformation. All the members of the family were confirmed to have no evidence of Peutz-Jeghers syndrome. Our clinical experience determines the malignant potency in Peutz-Jeghers and recommends that intensive follow-up of gastrointestinal and extragastrointestinal sites is needed in patients with this syndrome.