Cancer in Peutz-Jeghers syndrome

Cancer. 1993 Nov 1;72(9):2777-81. doi: 10.1002/1097-0142(19931101)72:9<2777::aid-cncr2820720940>;2-6.


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Duodenal Neoplasms / complications
  • Fatal Outcome
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Neoplasms / complications*
  • Ovarian Neoplasms / complications
  • Pancreatic Neoplasms / complications
  • Peutz-Jeghers Syndrome / complications*
  • Peutz-Jeghers Syndrome / physiopathology
  • Stomach Neoplasms / complications
  • Uterine Neoplasms / complications