ConditionDefinitionPrevalenceAverage age at symptomatic presentationAverage age at diagnosisWhen to start screeningScreening intervalRecommending organizationsComments
Attenuated familial adenomatous polyposis4,5 10 to 99 synchronous advanced adenomasUnknown52 years58 years (range = 29 to 81 years); 69% of patients studied developed CRC by 80 years of ageLate teens to mid-20sColonoscopy every one to two yearsACG, NCCNProximal colonic distribution necessitates colonoscopy for all screening
Familial adenomatous polyposis4,6,7 ≥ 100 colorectal adenomasApproximately three cases per 100,00036 years (range = 4 to 72 years)39 years; risk of developing CRC by 45 years of age is 87%Sigmoidoscopy starting at 10 to 12 years of ageSigmoidoscopy every one to two years until polyp is found, then colonoscopyACG, NCCNIf colectomy is delayed more than one year after polyps are found, colonoscopy should be performed annually
Hereditary nonpolyposis colorectal cancer6,8,9 May be defined clinically or by presence of one of five DNA mismatch repair genesUnknown but estimated to be one in 440< 45 years45 years; lifetime risk of CRC is 75% to 80%25 years of age or five years earlier than the first cancer case in the family, whichever comes firstColonoscopy every one to two yearsInternational Collaborative Group on HNPCC; Revised Bethesda Guidelines for HNPCC (National Cancer Institute); European Hereditary Tumour GroupSimplified clinical screening criteria:
Patients with a first-degree relative with CRC diagnosed before 50 years of age
Presence of synchronous and/or metachronous CRC or other HNPCC-associated tumors (e.g., endometrial or gastric)
Inflammatory bowel disease1013 Ulcerative colitis or Crohn disease with colonic involvementCrohn disease: 11 per 100,000
Ulcerative colitis: 12 per 100,000
Crohn disease: 30 years
Ulcerative colitis: 35 years
40 to 50 years; 4% to 5% lifetime risk of CRCColonoscopy eight to 10 years after the onset of symptomsEvery one to three yearsCrohn's & Colitis Foundation; American Cancer Society Colorectal Cancer Advisory groupAnnual surveillance colonoscopy in patients with primary sclerosing cholangitis
MUTYH-associated polyposis4,6,9,14 Typically < 100 colorectal adenomasLess than one in 10,000Unknown48 years; risk of CRC is 19% by 50 years of age and 43% by 60 years of ageColonoscopy beginning in late teens to mid-20sOne to two yearsACG, NCCNFirst described in 2002
Peutz-Jeghers syndrome4,6 Hamartomatous polyposisOne in 50,000 to 200,000Polyp growth begins in the first decade of life, but patients typically do not develop symptoms until the second or third decadeUnknownEsophagogastroduodenoscopy, colonoscopy, and video capsule endoscopy should begin at eight years of age and, if negative, be repeated every three yearsIf polyps, colonoscopy every three years If no polyps, wait until 18 years of age, then colonoscopy every three years; repeat video capsule endoscopy every three yearsACG, NCCNIntussusception is a common complication typically occurring in individuals younger than 20 years
Sessile serrated adenomatous polyposis15,16 At least five serrated polyps proximal to the sigmoid colon, two of which are greater than 10 mm in diameter
Any number of serrated polyps occurring proximal to the sigmoid colon in an individual who has a first-degree relative with serrated polyposis
More than 20 serrated polyps of any size distributed throughout the colon
Unknown but estimated to be one in 2,000 to 3,00044 to 62 years44 to 62 years; 25% to 70% have CRC at the time of diagnosisColonoscopy in first-degree relatives of patients with sessile serrated adenomatous polyposis syndrome beginning at 40 years of age or 10 years younger than the age at diagnosis of the youngest affected relativeAnnual colonoscopy with intent to clear proximal colon of all serrated lesions Colonoscopy every five years in first-degree relativesACGSurgery is indicated when CRC is diagnosed or polyps cannot be controlled endoscopically Following resection, endoscopic surveillance of any residual colon and rectum should be performed every six to 12 months