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 |
|
|
|
|
SMR, 95% CI |
SMR by treatment for retinoblastoma |
|
| 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 |
|
| 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 |
Abbreviations: O = observed number of soft tissue sarcomas; E = expected number of soft tissue sarcomas; CI = confidence intervals; AER = absolute excess risk per 10,000 persons, yr = year; SMR = standardized mortality ratio; NA not available.