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Cancer in Peutz-Jeghers syndrome
K Hizawa1, M Iida, T Matsumoto
1Second Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Background:
Peutz-Jeghers (P-J) syndrome has been found to be associated with an increased risk of malignant neoplasia.
Methods:
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.
Results:
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.
Conclusion:
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.