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      Disease expression in Swiss hereditary non-polyposis colorectal cancer (HNPCC) kindreds.

      1 , , ,
      International journal of cancer

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          Abstract

          The genetics of Hereditary Non-Polyposis Colorectal Cancer (HNPCC) has recently been established and found to be associated with DNA mismatch repair deficiency. As the molecular basis of this syndrome does not appear to predict any particular disease, we compared families selected according to the "Amsterdam" criteria (AC) against families that were selected because of an aggregation of colonic and extracolonic malignancies (EC), all of which have been observed in HNPCC families. A comparison of the 2 groups revealed that there were significant differences between them. Age at disease onset for both groups was 20-30 years younger than in the general population; however, a normal age distribution was observed for the AC group whereas for the EC group a bimodal distribution was apparent. The prognosis for both groups together did not differ from that of the general population; however, if split, the AC group had a significantly better outcome than the EC group. Furthermore, dividing the AC group into hMSH2- and hMLH1-linked families revealed that there was no difference in severity of disease between these 2 groups with respect to survival and mean age of disease onset. Both the AC and EC groups displayed a similar tumour spectrum with a virtually identical tumour distribution. A significant finding was the over-representation, of brain tumours in this family set, which comprised the third most common malignancy after endometrial and stomach cancer.

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          Author and article information

          Journal
          Int. J. Cancer
          International journal of cancer
          0020-7136
          0020-7136
          Jun 20 1997
          : 74
          : 3
          Affiliations
          [1 ] Human Genetics, Department of Research, University Hospital, Basel, Switzerland.
          Article
          10.1002/(SICI)1097-0215(19970620)74:3<281::AID-IJC8>3.0.CO;2-V
          9221805
          cc9beb09-d319-4961-b6e6-7fed539dd1d4
          History

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