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.
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.
Authors: Jason D Fraser; Steven E Briggs; Shawn D St Peter; Giovanni De Petris; Jacques Heppell Journal: Fam Cancer Date: 2008-08-23 Impact factor: 2.375
Authors: M M Entius; A M Westerman; F M Giardiello; M L van Velthuysen; M M Polak; R J Slebos; J H Wilson; S R Hamilton; G J Offerhaus Journal: Gut Date: 1997-09 Impact factor: 23.059
Authors: Chongjuan Wei; Christopher I Amos; Nianxiang Zhang; Jing Zhu; Xiaopei Wang; Marsha L Frazier Journal: Cancer Lett Date: 2009-01-14 Impact factor: 8.679