Abstract
Background : Peutz-Jeghers syndrome, a hereditary gastrointestinal polyposis with mucocutaneous pigmentation, puts its sufferers at 3% to 24% risk for cancer including sex cord tumor with annular tubules of the ovary, well-differentiated adenocarcinoma (adenoma malignum) of the uterine cervix, and breast cancer.
Case : A 39-year-old woman hospitalized for melena, abdominal pain, and fever had a long history of Peutz-Jeghers syndrome with repeated episodes of intestinal obstruction since childhood. Examination showed ascites and bilateral pleural effusion cytologically positive for adenocarcinoma cells containing intracytoplasmic mucin. Upper and lower gastrointestinal endoscopy showed polyposis consistent with Peutz-Jeghers syndrome and irregular genital bleeding. Abdominal computed tomography (CT) showed swelling of the uterine cervix suggestive of cervical cancer, confirmed by a Pap smear and uterine cervical biopsy to be endocervical mucinous adenocarcinoma. She died about a month later.
Conclusion : Adenocarcinoma of the uterine cervix should be considered in differential diagnosis when mucinous adenocarcinoma cells are encountered in effusion cytology specimens from those with Peutz-Jeghers syndrome.
