Table 1.
Summary of bone sarcoma after retinoblastoma in cohort studies of 100 or more hereditary retinoblastoma survivors
| Study | Study design, Years of Rb diagnosis | No. subjects with hereditary retinoblastoma | Years of follow-up: median/mean | No. bone sarcoma cases | O/E, 95% CI | O/E by treatment for retinoblastoma | Comments |
|---|---|---|---|---|---|---|---|
|
1a. Incidence | |||||||
| Kleinerman 2005
[7] US Two US medical centers |
Hospital-based 1914-1984 |
963 1-yr survivors |
Mean: 25 |
75 |
360 (283–451) |
Any radiation: 406 (318–511) No radiation: 69 (8.4-250) Radiation + chemotherapy: 539 (384–733); Radiation, no chemotherapy: 302 (205–428) |
AER = 29.6 |
| Reulen 2011
[16] British Childhood Cancer Survivor Study, UK |
Population-based 1940-1991 |
NA*, 5-yr survivors |
Mean: 26 |
35 |
289 (209–402) |
NA |
AER = 23; * No. of Rb survivors not given but there are estimated to be 809 hereditary Rb subjects based on MacCarthy et al.
[44] |
| Marees 2008
[6] Netherlands Dutch Retinoblastoma Registry |
Registry -based 1945-2005 |
298 survivors |
Median: 22 |
16 |
314 (180–511) |
Radiation only: 302 (130–596) Radiation + chemotherapy: 586 (215–1275); Surgery only: 75 (1.9-421) |
AER = 23 |
| Tucker 1987
[14] US Late Effects Study Group |
Hospital-based 1945-1979 |
319 2-yr survivors |
Mean: 7 |
12 |
999 (515–1745) |
|
*Hereditability not specified |
|
1b. 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 |
56 |
595 (449–773) |
Radiation: 673 (506–879) |
AER = 19.8; *No difference between males and females |
| Marees 2009
[46] Netherlands Dutch Retinoblastoma Registry |
Registry-based 1862 - 2005 |
337 (alive in 1961) |
Median: 26 yr Follow-up 1961-2005 |
11 |
289 (144–517) |
Radiation only: 266 (72.2-680) Radiation + chemotherapy: 659 (179–1686); Surgery only: 124 (15–449) |
Majority deaths from bone cancer occur within first 30 years |
| Acquaviva 2006
[46] Italian Retinoblastoma Registry |
Registry-based 1923-2003 |
408 |
Median: 11 |
9 |
392 (204-753) |
NA |
|
| Fletcher 2004 [10] Patients from British hospitals and linkage with national registry | Hospital-based 1873-1950 | 144 25-yr survivors | Follow up began in 1940 Median age: 60 | 1 | 32.4 (0.82 - 180) | NA | *Radiation was not typically used to treat Rb during these years |
Abbreviations: O = observed number of bone sarcomas; E = expected number of bone sarcomas; CI = confidence intervals; AER = absolute excess risk per 10,000 persons, yr = year; SMR = standardized mortality ratio; NA not available.