Table 2 Summary of soft tissue sarcoma after retinoblastoma in cohort studies of 100 or more hereditary retinoblastoma survivors
Study | Study Design Years of Rb diagnosis | Number of subjects with hereditary retinoblastoma | Years of follow-up: median/mean | No. of Soft tissue sarcomas | O/E, 95% CI | O/E by treatment for retinoblastoma | Comments |
|---|---|---|---|---|---|---|---|
2a. Incidence | |||||||
Kleinerman 2007[22] US Two medical centers | Hospital-based 1914-1984 | 963 1-yr survivors | Mean: 25 | 69 | 184 (143–233) | Any radiation: 212 (164–270); No radiation: 47 (9.4-137); Any chemotherapy: 236 (161–333); No chemotherapy: 193 (133–271) | AER = 27 *No evidence of risk modification by sex *SIRs highest within first 10 years but remained significantly elevated ≥30 |
Reulen 2011[16] British Childhood Cancer Survivor Study | Population-based 1940-1991 | NA, 5-yr survivors | Mean: 26 | 16 | N/A | N/A | Rates increase over time since Rb (highest >25) |
Marees 2008[6] Netherlands Dutch Retinoblastoma Registry | Registry-based 1945-2005 | 298 | Median: 22 | 20 | 243 (148–375) | Radiation only: 303 (161–517) Radiation + chemotherapy: 354 (129–770) Surgery only: 48.4 (1.23-270) | AER = 29; SIRs elevated in all time periods (3 cases ≥40) |
Tucker 1987[14] US Late Effects Study Group | Hospital-based 1945-1979 | 319 2-yr survivors (hereditability not specified) | Mean: 7 | 4 | 235 (64–602) |  | All cases observed among females |
2b. Mortality | Â | Â | Â | Â | SMR, 95% CI | SMR by treatment for retinoblastoma | Â |
Yu 2009[12] US Two medical centers | Hospital-based 1914-1996 | 1092 1-yr survivors | Median: 29 | 31 | 329 (223–467) | Any Radiation 395 (268–560) | AER = 10.9; SMR is higher for women vs men (not statistically significant) |
Marees 2009[45] Netherlands Dutch Retinoblastoma Registry | Registry-based 1862 - 2005 | 337 | Median: 26 Follow-up 1961-2005 | 13 | 276 (147–472) | Radiation only: 311 (101–725) Rad + chemotherapy: 940 (345–2064); Surgery only: 85.2 (10.3-308) | *Deaths observed up to ≥50 years after RB *SMR peaks at 20–29 years but SMRs significantly elevated in all time periods |
Acquaviva 2006[46] Italian Retinoblastoma Registry | Registry-based 1923-2003 | 408 | Median: 12 | 6 | 453 (203.5 - 1008) | NA | Â |
Fletcher 2004[10] UK Patients from British hospitals; linkage with national registry | Hospital-based 1873-1950 | 144 25-yr survivors | Median attained age: 60; Follow-up began in 1940 | 4 | 110 (29–281) | NA | *Treatment not available, but radiation was not typically used during these years of Rb diagnosis |