Four patients with an established diagnosis of
Cowden's disease underwent
barium meal study, upper gastrointestinal endoscopy, barium enema examination, and colonoscopy. In three, the esophagus was affected by small protrusions, which were diagnosed as glycogenic acanthosis. Numerous hyperplastic
polyps were found in the stomach in three patients, and in one an inflammatory
fibroid polyp was also detected. Either lymphangiectasia or lymphoid
polyps were found in the duodenum in two patients. In all patients, the colon showed
polyps that varied in histology and included
adenoma, hamartomatous
polyp, and ganglioneurofibroma. In addition, jejunal
lymphangiomas were found in one of the three patients in whom the small intestine could be precisely evaluated. These findings suggest that the gastrointestinal involvement in
Cowden's disease is characterized by various benign lesions, especially esophageal glycogenic acanthosis, numerous gastric hyperplastic
polyps, and multiple hamartomatous
polyps in the rectosigmoid colon. Detection of these gastrointestinal manifestations may lead to early diagnosis of this potentially malignant disease.