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Table 1.

Syndromes leading to an increased risk of second colorectal malignancies

Syndromes with adenomatous polyps 
    APC gene mutations (1%) 
        Familial adenomatous polyposis 
        Attenuated adenomatous polyposis coli 
        Turcot's syndrome (two thirds of families) 
    MMR gene mutations (3%) 
        Hereditary nonpolyposis colorectal cancer types I and II 
        Muir-Torre syndrome 
        Turcot's syndrome (one third of families) 
Syndromes with hamartomatous polyps (<1%) 
    Peutz-Jeghers (LKB1
        Juvenile polyposis (SMAD4, PTEN
        Cowden (PTEN
            Bannayan-Ruvalcaba-Riley syndrome 
        Mixed polyposis 
Other familial causes (up to 20-25%) 
    Family history of adenomatous polyps (MYH
    Family history of colon cancer 
        Risk >3-fold if two first-degree relatives or one first-degree relative <50 years with colon cancer 
        Risk 2-fold if second-degree relative affected 
    Familial colon-breast cancer 
Nonfamilial causes 
    Personal history of adenomatous polyps 
    Personal history of colorectal cancer 
    Inflammatory bowel disease (ulcerative colitis, Crohn's colitis) 
    Radiation colitis 
    Ureterosigmoidostomy 
    Acromegaly 
    Cronkhite-Canada syndrome 
Syndromes with adenomatous polyps 
    APC gene mutations (1%) 
        Familial adenomatous polyposis 
        Attenuated adenomatous polyposis coli 
        Turcot's syndrome (two thirds of families) 
    MMR gene mutations (3%) 
        Hereditary nonpolyposis colorectal cancer types I and II 
        Muir-Torre syndrome 
        Turcot's syndrome (one third of families) 
Syndromes with hamartomatous polyps (<1%) 
    Peutz-Jeghers (LKB1
        Juvenile polyposis (SMAD4, PTEN
        Cowden (PTEN
            Bannayan-Ruvalcaba-Riley syndrome 
        Mixed polyposis 
Other familial causes (up to 20-25%) 
    Family history of adenomatous polyps (MYH
    Family history of colon cancer 
        Risk >3-fold if two first-degree relatives or one first-degree relative <50 years with colon cancer 
        Risk 2-fold if second-degree relative affected 
    Familial colon-breast cancer 
Nonfamilial causes 
    Personal history of adenomatous polyps 
    Personal history of colorectal cancer 
    Inflammatory bowel disease (ulcerative colitis, Crohn's colitis) 
    Radiation colitis 
    Ureterosigmoidostomy 
    Acromegaly 
    Cronkhite-Canada syndrome 

NOTE: Modified with permission from Libutti et al. (2).

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