Other Rare Polyposis Syndromes

The rare gastrointestinal polyposis syndromes are a group of disorders characterized by the presence of multiple polyps in the gastrointestinal tract, associa- ted in some cases with extraintestinal manifestations. These syndromes can be inherited or non-inherited. The first group includes, on the basis of polyp histopathology, adenomatous polyposis (familial adenomatous polyposis (FAP) vari- ants like Gardner's syndrome and Turcot's syndrome) and hamartomatous polyposis (Juvenile Polyposis syndrome, Peutz-Jeghers syndrome, Cowden's disease, and Bannayan-Ryley-Ruvalcaba syndrome), while the second group includes Cronkhite-Canada syndrome and Hyperplastic polyposis. The molecular genetics have not been well defined for all the syndromes. However, these conditions present variable risk of gastrointestinal and extraintestinal invasive malignancy. Therefore management and surveillance programs are different for each syndrome.