Table 2.
Characteristics of hereditary cancer syndromes by population prevalence, proband's health status and frequency of index cancers among blood relatives.
| Diagnosis | Population prevalence | Affected probands | Cancer frequency | |||
|---|---|---|---|---|---|---|
| Value,% | 95% CI,% | Fr,% | 95% CI,% | Value,% | 95% CI,% | |
| HNPCC/g1 | 0.06 | 0.03 - 0.10 | 18.2 | 5.1 - 47.7 | 30.1 | 23.3 - 38.0 |
| 15.8CRC | 10.7 - 22.5CRC | |||||
| 22.4EF | 14.8 - 32.3EF | |||||
| HNPCC/g2 | 0.11 | 0.07 - 0.17 | 5 | 0.9 - 23.6 | 15.5 | 11.6 - 20.3 |
| 10.6CRC | 7.4 - 14.8CRC | |||||
| 9.6EF | 5.7 - 15.8EF | |||||
| HNPCC/g3 | 0.17 | 0.12 - 0.24 | 9.7 | 3.3 - 24.9 | 19.3 | 16.5 - 22.5 |
| 12.4CRC | 9.6 - 15.9CRC | |||||
| 14.5EF | 10.5 - 19.8EF | |||||
| FCC/g2 | 0.11 | 0.07 - 0.17 | 5 | 0.9 - 23.6 | 17.0 | 12.8 - 22.3 |
| HB+OC/g1 | 0.03 | 0.01 - 0.06 | 20 | 3.6 - 62.4 | 24.6 F | 15.5 - 36.7 F |
| HBC1/g2 | 0.63 | 0.52 - 0.75 | 4.3 | 1.8 - 9.6 | 8.6 | 7.2 - 10.2 |
| 16.3F | 13.8 - 19.1F | |||||
| HBC2/g2 | 0.34 | 0.27 - 0.44 | 18.8 | 11.1 - 30.0 | 31.8F | 27.5 - 36.4F |
| HBOC1/g2 | 0.03 | 0.02 - 0.07 | 0 | 0 - 39.0 | 19.3F | 9.2 - 36.3 F |
| HBOC2/g2 | 0.16 | 0.11 - 0.22 | 20.7 | 9.8 - 38.4 | 30.8F | 25.0 - 37.3F |
| HOC/g2 | 0.008 | 0.001 - 0.06 | 0 | 0 - 49.0 | 36.4F | 19.7 - 57.0F |
| HB+OC/g2 | 1.18 | 1.04 - 1.35 | 10.5 | 7.1 - 15.2 | 12.6 F | 11.4 - 13.9 F |
| 23.6F | 21.4 - 25.9F | |||||
| HEC/g1 | 0.03 | 0.01 - 0.06 | 0 | 0 - 43.3 | 41.5F | 27.8 - 56.6F |
| HEC/g2 | 0.14 | 0.10 - 0.20 | 15.4 | 6.2 - 33.5 | 32.2F | 25.7 - 39.4 F |
| FEC/g1 | 0.01 | 0.003 - 0.04 | 0 | 0 - 65.8 | 46.2 F | 23.2 - 70.9 F |
| FEC1/g2 | 0.13 | 0.09 - 0.19 | 12.5 | 4.3 - 31.0 | 28.7 F | 22.4 - 36.0 F |
| FEC2/g2 | 0.05 | 0.03 - 0.09 | 11.1 | 2.0 - 43.5 | 32.4 F | 22.4 - 44.2 F |
| H+FEC/g1 | 0.04 | 0.02 - 0.08 | 0 | 0 - 35.4 | 42.6 F | 36.3 - 55.8 F |
| H+FEC/g2 | 0.32 | 0.25 - 0.41 | 13.6 | 7.0 - 24.5 | 30.8 F | 26.5 - 35.4 F |
| H+FEC g3 | 0.35 | 0.28 - 0.45 | 14.3 | 7.4 - 25.7 | 41.0 F | 36.1 - 46.2 F |
| HGC/g1 | 0.11 | 0.07 - 0.17 | 4.8 | 0.8 - 22.7 | 25.2 | 20.6 - 30.4 |
| HGC/g2 | 0.40 | 0.32 - 0.50 | 0 | 0 - 4.9 | 16.0 | 13.8 - 18.5 |
| HGC/g3 | 0.51 | 0.42 - 0.62 | 1.1 | 0.2 - 5.7 | 18.2 | 16.2 - 20.5 |
| FLC/g1 | 0.07 | 0.04 - 0.12 | 0 | 0 - 22.8 | 25.5 | 19.3 - 32.8 |
| FLC/g2 | 0.50 | 0.41 - 0.61 | 0 | 0 - 4.0 | 17.2 | 15.0 - 19.7 |
| FLC/g3 | 0.57 | 0.47 - 0.69 | 0 | 0 - 3.5 | 18.3 | 16.2 - 20.7 |
| HPC/g1 | 0.005 | 0.001 - 0.03 | 100 | 20.7 - 100 | 21.4M | 7.6 - 47.6M |
| HPC/g2 | 0.11 | 0.07 - 0.17 | 19.0 | 7.7 - 40.0 | 22.2M | 16.4 - 29.4M |
| HPC/g3 | 0.12 | 0.08 - 0.18 | 22.7 | 10.1 - 43.4 | 22.2 | 16.5 - 29.0 |
| FPanC/g1 | 0.05 | 0.03 - 0.10 | 10 | 1.8 - 40.4 | 14.7 | 9.1 - 22.9 |
| FBlaC/g1 | 0.01 | 0.003 - 0.04 | 0 | 0 - 65.8 | 31.6 | 15.4 - 54.0 |
| FBlaC/g2 | 0.05 | 0.03 - 0.09 | 0 | 0 - 29.9 | 20.0 | 11.8 - 31.8 |
| FBlaC/g3 | 0.06 | 0.03 - 0.11 | 0 | 0 - 25.9 | 22.8 | 14.9 - 33.2 |
| FHemT/g1 | 0.005 | 0.001 - 0.03 | 0 | 0 - 79.3 | 30.8 | 12.7 - 57.6 |
| FHemT/g2 | 0.09 | 0.05 - 0.14 | 6.3 | 1.1 - 28.3 | 15.4 | 11.2 - 20.9 |
| FHemT/g3 | 0.09 | 0.06 - 0.15 | 5.9 | 1.0 - 27.0 | 16.3 | 12.1 - 21.2 |
| FBT/g1 | 0.02 | 0.005 - 0.05 | 0 | 0 - 56.2 | 32.3 | 18.6 - 49.9 |
| FBT/g2 | 0.09 | 0.05 - 0.14 | 0 | 0 - 19.4 | 14.4 | 10.4 - 19.5 |
| FBT/g3 | 0.10 | 0.07 - 0.16 | 0 | 0 - 16.8 | 16.5 | 12.5 - 21.5 |
CRC colorectal cancer; EF endometrial cancer in female; F in female; M in male
CI, confidence interval; Fr, frequency; HB+OC, combined group of hereditary breast and/or ovarian cancer; H+FEC, combined group of hereditary and familial endometrial cancer