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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