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

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