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Table 1 Summary of bone sarcoma after retinoblastoma in cohort studies of 100 or more hereditary retinoblastoma survivors

From: Sarcomas in hereditary retinoblastoma

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
  1. 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.