Table 2.

Clinical features and colon cancer risk in Hamartomatous Polyposis Syndromes, according to literature series

Syndrome	Main clinical features polyp distribution	Increased risk of other tumors	Colon cancer risk
Juvenile Polyposis	Juvenile polyps Distribution: large bowel (mainly), small bowel, stomach	Gastric and colorectal	39%-68%
Peutz-Jeghers	Peutz-Jeghers polyps Typical melanotic oral and dermic pigmentations Distribution: small bowel, large bowel, stomach	Gastric, small bowel, pancreas, colorectal, ovary, uterus, breasts, sex cords	39%-57%
PTEN	Mucocutaneous tumors (multiple trichilemmomas) Distribution: Small bowel, large bowel, stomach	Breast, thyroid, retina and uterus cancer	18%