• We are sorry, but NCBI web applications do not support your browser and may not function properly. More information
Logo of annsurgLink to Publisher's site
Ann Surg. Sep 1987; 206(3): 289–295.
PMCID: PMC1493182

Recognition and treatment of patients with hereditary nonpolyposis colon cancer (Lynch syndromes I and II).


Primary genetic factors are etiologic in at least 5-10% of patients with colon cancer. The polyposis syndromes (FPC) are easily identified examples because of the spectacular number of polyps. The hereditary nonpolyposis syndromes (HNPCC), although five times more common than FPC, are usually not recognized because they do not have such a distinctive clinical, premonitory genetic marker. Colorectal cancer expression was surveyed in 10 extended, thoroughly documented HNPCC kindreds. One hundred sixteen patients were found to have 183 colorectal cancers. Despite the striking family history, less than 5% were correctly treated by subtotal colectomy. This provided a unique opportunity to study the natural history. Five findings differed significantly (p less than 0.05) from patients with sporadic colon cancer: (1) mean age of initial colon cancer diagnosed was 45.6 years; (2) 69.1% of first colon cancers were located proximal to the splenic flexure of the colon; (3) 18.1% had synchronous colon cancer; (4) 24.2% had metachronous colon cancer develop with life table analysis showing the risk for a metachronous lesion at 10 years to be 40%; and (5) only 23.3% of cancers were located in the sigmoid colon or rectum. Based on this data, it is recommended that the family history of all patients with a newly diagnosed colon cancer be evaluated for evidence of this syndrome. If an autosomal dominant inheritance pattern emerges, an in-depth genetic investigation is indicated. When HNPCC is confirmed, the following recommendations apply: a subtotal abdominal colectomy is indicated at the time of the initial colon cancer because of the risk of synchronous and metachronous lesions. The rectum should be spared in favor of careful lifetime surveillance because of the proclivity for proximal colon cancer involvement. As yet unaffected members of a newly diagnosed HNPCC kindred who are in the "direct genetic line" should be cautioned that they are at 50% risk and must begin an intensive surveillance program beginning in the third decade with careful attention to the right colon. Patients from newly diagnosed HNPCC families who have had a previous conventional colectomy for colon cancer should, at the very least, enter an intensive surveillance program; a prophylactic completion subtotal colectomy should be considered for patients who are less than totally compliant.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (1.0M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Silverberg E, Lubera J. Cancer statistics, 1987. CA Cancer J Clin. 1987 Jan-Feb;37(1):2–19. [PubMed]
  • 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 May;28(5):310–316. [PubMed]
  • Falterman KW, Hill CB, Markey JC, Fox JW, Cohn I., Jr Cancer of the colon, rectum, and anus: a review of 2313 cases. Cancer. 1974 Sep;34(3):suppl–suppl:959. [PubMed]
  • Langevin JM, Nivatvongs S. The true incidence of synchronous cancer of the large bowel. A prospective study. Am J Surg. 1984 Mar;147(3):330–333. [PubMed]
  • Reilly JC, Rusin LC, Theuerkauf FJ., Jr Colonoscopy: its role in cancer of the colon and rectum. Dis Colon Rectum. 1982 Sep;25(6):532–538. [PubMed]
  • Dozois RR. Restorative proctocolectomy and ileal reservoir. Mayo Clin Proc. 1986 Apr;61(4):283–286. [PubMed]
  • Lynch HT, Albano WA, Ruma TA, Schmitz GD, Costello KA, Lynch JF. Surveillance/management of an obligate gene carrier: the cancer family syndrome. Gastroenterology. 1983 Feb;84(2):404–408. [PubMed]
  • Lynch HT, Albano WA, Lynch JF, Lynch PM, Campbell A. Surveillance and management of patients at high genetic risk for ovarian carcinoma. Obstet Gynecol. 1982 May;59(5):589–596. [PubMed]

Articles from Annals of Surgery are provided here courtesy of Lippincott, Williams, and Wilkins


Related citations in PubMed

See reviews...See all...

Cited by other articles in PMC

See all...


  • MedGen
    Related information in MedGen
  • PubMed
    PubMed citations for these articles

Recent Activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...