Cancer in peutz-jeghers syndrome

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Abstract
Peutz-Jeghers (P-J) syndrome has been found to be associated with an increased risk of malignant neoplasia. The authors reviewed the clinical courses of eight patients with P-J syndrome (four male and four female patients, ranging in age from 8 to 55 years), who had been followed up for as long as 12 years. The diagnosis of this syndrome was based on the evidence of characteristic mucocutaneous pigmentations and gastrointestinal hamartomatous polyposis. Four cases of malignant neoplasm among the eight patients were found. In a 25-year-old woman, an extremely well-differentiated adenocarcinoma of the uterus cervix was found at the initial examination. A gastric cancer in a 32-year-old woman, duodenal cancers in a 43-year-old man, and a pancreatic cancer in a 60-year-old man also were identified 12, 10, and 5 years after the time of initial examinations, respectively. Two of these patients died of the effects of the tumor soon after. The authors' experience confirms a veritable malignant potency in P-J syndrome and suggests that an intensive follow-up of gastrointestinal and extra-gastrointestinal sites is needed in patients with this syndrome.