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The hereditary nonpolyposis colon cancer (HNPCC) is the most common form of hereditary colorectal cancer. Also called familial adenomatous polyposis (FAP), is an autosomal dominant genetic condition characterized by the presence of multiple polyps (> 100) or multiple adenomas in the large intestine. There is a 100% chance of developing CRC in patients with a germline mutation in the APC gene. This cancer is called Gardner’s syndrome when presents extracolonic demonstrations, or Turcot syndrome if they are at the central nervous system level.