LYNCH SYNDROME AND SPORADIC COLORECTAL CANCER: CLINICAL AND GENEALOGICAL FEATURES

Abstract

Two hundred and one families that had cases of colorectal cancer among first-degree relatives and had been followed up for at least 5 years were selected from the Moscow Familial Cancer Registry and divided into 3 groups: 1) 6 families with Lynch syndrome (hereditary nonpolyposis colorectal cancer); 2) 36 cancer families without Lynch syndrome; 3) 159 noncancer families. Comparative analysis has shown that the families with Lynch syndrome significantly differ from those of two other subgroups in the following respects: 1) hereditary cancer loading (cancer cases in more than 60% of relatives over 20 years of age); 2) high incidence rates for multiple cancers in women; 3) more common cancer involvement of the colon than the rectus; 4) cancer of the corpus uteri is a second malignancy after colorectal cancer among women; 5) primary malignancies occur 10-20 years earlier with the tumor-specific survival being 5-7 years longer than in the two other groups; 6) during a 5-year follow-up, new cancer cases occurred among first-degree and first-to-third-degree relatives in 50 and 83% of the families, respectively. The cancer families differed from noncancer ones only in general hereditary cancer loading (35.6 versus 12.5%) and in the rate of new cases in first-to-third relatives (33.3% versus 10.7%).

Keywords

Lynch syndrome / hereditary nonpolyposis colorectal cancer / cancer families

References

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