Summary of Chapter 3: Identification, Surveillance and Management of Hereditary Non-polyposis Colorectal Cancer

Abstract

The first presentation (#8) of the second section of workshop A2 was given by Hans F. A. Vasen of the Netherlands, and entitled “Screening for hereditary nonpolyposis colorectal cancer in the Netherlands.” The investigators compared the stage of colon cancers in 97 patients who belonged to “Lynch” syndrome families and presented with symptoms, to 20 relatives in these families who were screened while asymptomatic. The symptomatic group was found to have more advanced stage tumors than the screened group. Symptomatic group: Dukes A, 6 patients; Dukes B, 43 patients; Dukes C, 24 patients; Dukes D, 11 patients; unclassified, 13 patients. Screened group: adenomas only, 14 patients; Dukes A, 2 patients; Dukes B, 4 patients. The authors noted other features of the Lynch syndromes including early age onset of cancer (mean age 46 years), 60% proximal tumors, 25% metachronous tumors and an excess of endometrial, stomach and urinary-tract tumors. They recommended that periodic colonoscopic screening begin at age 20 years and that subtotal colectomy be performed with the first diagnosis of colon cancer.