Table 1

Age at diagnosis and death and cause of death in individuals with inherited FAP over two time spans

Pre-RegistryPost-Registry
1969–1988 symptomatic1969–1988 screened1989–2009 symptomatic1989–2009 screened
Number474046140
Median age at diagnosis (range)35 (1–62)17 (7–59)39 (6–76)14* (1–62)
Died29 (61.7%)5 (12.5%)17(37%)4(2.9%)
Cause of death CRC21 (44.7%)2 (5%)13 (28.3)0
Cause of death desmoids1 (2.1%)1 (2.5%)1(2.2%)3 (2.1%) Ages 21, 25, 45
Cause of death FAP-related cancer (eg, duodenal, thyroid)4 (8.5%)
1 duodenal
1 pancreatic
1 oesophageal
1 hepatoblastoma
01 gastric (2.2%)1 thyroid (0.7%)
Cause of death other3 (6.4%)2 (5%)20
  • * 64 diagnosed on DNA testing aged 9–12 years (three individuals diagnosed on DNA testing before 9 years).

  • CRC, colorectal cancer; FAP, familial adenomatous polyposis.