Lynch syndrome and familial adenomatous polyposis represent the large majority of hereditary colorectal cancers; however, several additional less common syndromes are also associated with increased risk for developing malignancy. Identification of these syndromes requires an understanding of the phenotype to recognize the disease. Proper management of individuals and families affected by these syndromes provides an opportunity for cancer prevention. The purpose of this article is to provide a concise overview of the hereditary polyposis and colorectal cancer syndromes, exclusive of familial adenomatous polyposis and hereditary nonpolyposis colorectal cancer.
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