Life‐time risk of different cancers in hereditary non‐polyposis colorectal cancer (hnpcc) syndrome

摘要: Identification of hereditary non-polyposis colorectal cancer (HNPCC) indicates theoretical life-time risks 50% for the descendants an affected family member and 100% true gene carriers. However, besides (CRC), many other types sites are also involved, which gives reason to evaluate magnitude risk various types. A detailed pedigree analysis 40 families with HNPCC identified 414 patients cancer. Kaplan-Meier life-table cumulative cancers was performed on basis 293 putative carriers who had adequate clinical histological documentation their tumors. Cumulative were highest (78%) endometrial (43%, women only), followed by gastric, biliary tract, urinary tract ovarian (19-9%). For probably HNPCC-related types, such as small bowel carcinoma brain tumors, only 1%. The any metachronous reached 90% after treatment CRC 75% cancer; second tumor most often a new or remains important type in syndrome but does not develop all This makes decision possible prophylactic colectomy test-detected difficult. Of at least is common enough necessitate specific surveillance program. © 1995 Wiley-Liss, Inc.