Hereditary nonpolyposis colorectal cancer syndrome

What is hereditary nonpolyposis colorectal cancer syndrome (HNPCC)? 

  • HNPCC syndrome is an autosomal dominant disorder distinguished by the early development of CRC (average age is 44).
  • The diagnostic clinical criteria used to help establish the diagnosis of HNPCC include the Amsterdam (modified) and Bethesda classification systems.

What are the endoscopic screening and surveillance guidelines for HNPCC? 

  • Colonoscopy should be performed every 1 to 2 years in patients at risk for HNPCC, beginning at age 20 to 25 years or 10 years younger than the age of the earliest diagnosis of cancer in the family.
  • The screening interval for endoscopic surveillance changes to yearly beginning at age 40.

Do patients with ulcerative colitis (UC) and Crohn’s disease require endoscopic surveillance? 

  • Yes. Long-standing UC and extensive Crohn’s disease increase an individual’s risk for the development of dysplasia and CRC.
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