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The tumor spectrum in HNPCC

P Watson1, H T Lynch

  • 1Department of Preventive Medicine, Creighton University School of Medicine, Omaha, Nebraska 68178.

Anticancer Research
|July 1, 1994
PubMed
Summary

Hereditary Non-Polyposis Colorectal Cancer (HNPCC) increases risks for colorectal and endometrial cancers. This study found HNPCC also elevates risks for stomach, small bowel, ovarian, and other cancers, with varying risk levels among families.

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Area of Science:

  • Oncology
  • Genetics
  • Epidemiology

Background:

  • Hereditary Non-Polyposis Colorectal Cancer (HNPCC) is an inherited cancer syndrome.
  • Known risks include colorectal and endometrial cancers.
  • Potential risks for other extracolonic cancers and family-specific risk heterogeneity are hypothesized.

Purpose of the Study:

  • To evaluate the hypotheses of increased extracolonic cancer risk and risk heterogeneity in HNPCC families.
  • To identify specific extracolonic sites with elevated cancer risks in HNPCC individuals.
  • To assess the distribution of extracolonic cancer risks among different HNPCC families.

Main Methods:

  • A cohort study of 1317 high-risk members from 23 HNPCC families.
  • Comparison of observed cancer occurrences against expected numbers derived from general population incidence data.
  • Analysis of cancer distribution among families to test for heterogeneity against a uniform risk hypothesis.

Main Results:

  • Significant excesses of stomach, small bowel, hepatobiliary, upper urologic tract, and ovarian cancers were observed in HNPCC family members.
  • Gastrointestinal tract cancers showed uniform distribution across families.
  • Urologic and gynecologic tract cancers exhibited significant heterogeneity in risk distribution among families.

Conclusions:

  • HNPCC family members face elevated risks for specific extracolonic cancers beyond colorectal and endometrial sites.
  • Evidence supports heterogeneity in extracolonic cancer risk among different HNPCC families, particularly for urologic and gynecologic cancers.

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