Skip to main content

Table 2 Summary of soft tissue 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 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
  1. 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.