Abstract
Approximately 5 to 6 percent of the total colorectal cancer burden is accounted for by hereditary nonpolyposis colorectal cancer (HNPCC). Because clinical premonitory signs such as those seen in familial polyposis coli (FPC) are lacking, the clinician must recognize clinical findings and family history typical of HNPCC. The authors have described colorectal cancer expression from a survey of ten HNPCC kindreds. Kindred members with colorectal cancer differed significantly (P<.05) from patients with sporadic colorectal cancer: 1) mean age of initial colon cancer diagnosis was 44.6 years; 2) 72.3 percent of first colon cancers were located in the right colon, and only 25 percent were in the sigmoid colon and rectum; 3) 18.1 percent had synchronous colon cancers; and 4) 24.2 percent developed metachronous colon cancer, with a risk for metachronous lesions in ten years of 40 percent. Affecteds and their first-degree relatives should undergo early intensive education and surveillance. In families with an early age of onset, colonoscopy should begin at age 25, and biannually thereafter, with fecal occult blood testing of the stool semiannually. Third-party carriers must become more responsive to the costly surveillance measures required for these otherwise healthy patients.
Similar content being viewed by others
References
Lynch HT, Rozen P, Schuelke GS, Lynch JF. Hereditary colorectal cancer review: colonic polyposis and nonpolyposis colonic cancer (Lynch syndrome I and II). Surv Dig Dis 1984;2:244–60.
Lynch HT. Frequency of hereditary nonpolyposis colorectal carcinoma (Lynch syndromes I and II). Gastroenterology 1986;90:486–9.
Mecklin J-P. Frequency of hereditary colorectal carcinoma. Gastroenterology 1987;93:1021–5.
Lynch PM, Lynch HT. Colon cancer genetics. New York: VN Reinhold, 1985.
Lynch HT, Lynch PM, Albano WA, Lynch JF. The cancer family syndrome: a status report. Dis Colon Rectum 1981;24:311–22.
Lynch HT, Voorhees GJ, Lanspa SJ, McGreevy PS, Lynch JF. Pancreatic carcinoma and hereditary nonpolyposis colorectal cancer: a family study. Br J Cancer 1985;52:271–3.
Lynch HT, Kimberling W, Albano WA, et al. Hereditary nonpolyposis colorectal cancer (Lynch syndromes I and II). I Clinical description of resource. II. Biomarker studies. Cancer 1985;56:934–8; 939–51.
Lynch HT, Fitzgibbons R Jr, Marcus J, McGill J, Voorhees GJ, Lynch JF. Colorectal cancer in a nuclear family: familial or hereditary. Dis Colon Rectum 1985;28:310–6.
Boland CR, Troncale FJ. Familial colonic cancer without antecedent polyposis. Ann Intern Med 1984;100:700–1.
Cunliffe WJ, Hasleton PS, Tweedle DE, Schofield PF. Incidence of synchronous and metachronous colorectal carcinoma. Br J Surg 1984;71:941–3.
Reilly JC, Rusin LC, Theuerkauf FJ. Colonoscopy: its role in cancer of the colon and rectum. Dis Colon Rectum 1982;25:532–8.
Beart RW, Melton LJ III, Maruta M, Dockerty MB, Frydenberg HB, O'Fallon WM. Trends in right and left-sided colon cancer. Dis Colon Rectum 1983;26:393–8.
Mamazza J, Gordon PH. The changing distribution of large intestinal cancer. Dis Colon Rectum 1982;25:558–62.
Lynch PM, Lynch HT, Harris RE. Hereditary proximal colonic cancer. Dis Colon Rectum 1977;20:661–8.
Bodmer WF, Bailey CJ, Bodmer J, et al. Localization of the gene for familial adenomatous polyposis on chromosome 5. Nature 1987;328:614–6.
Solomon E, Voss R, Hall V, et al. Chromosome 5 allele loss in human colorectal carcinomas. Nature 1987;328:616–9.
Mecklin J-P, Sipponen P, Jarvinen HJ. Histopathology of colorectal carcinomas and adenomas in cancer family syndrome. Dis Colon Rectum 1986;29:849–53.
Alley PG, McNee RK. Age and sex differences in right colon cancer. Dis Colon Rectum 1986;29:227–9.
Eide TJ, Schweder T. Clustering of adenomas in the large intestine, Gut 1984;25:1262–7.
Rothberg PG, Spandorfer JM, Erisman MD et al. Evidence thatc-myc expression defines two genetically distinct forms of colorectal adenocarcinoma. Br J Cancer 1985;52:629–32.
Reichmann A, Levin B, Martin P. Human large-bowel cancer: correlation of clinical and histopathological features with banded chromosomes. Int J Cancer 1982;29:625–9.
Author information
Authors and Affiliations
Additional information
Supported by NIH Grants No. 5 RO1 CA41371-01 and 1 RO1 CA42705-01.
About this article
Cite this article
Lynch, H.T., Watson, P., Lanspa, S.J. et al. Natural history of colorectal cancer in hereditary nonpolyposis colorectal cancer (Lynch syndromes I and II). Dis Colon Rectum 31, 439–444 (1988). https://doi.org/10.1007/BF02552613
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF02552613