Chronic medical conditions in adult survivors of retinoblastoma: Results of the Retinoblastoma Survivor Study Journal Article


Authors: Friedman, D. N.; Chou, J. F.; Oeffinger, K. C.; Kleinerman, R. A.; Ford, J. S.; Sklar, C. A.; Li, Y.; McCabe, M. S.; Robison, L. L.; Marr, B. P.; Abramson, D. H.; Dunkel, I. J.
Article Title: Chronic medical conditions in adult survivors of retinoblastoma: Results of the Retinoblastoma Survivor Study
Abstract: BACKGROUND Limited data are available regarding long-term morbidity in adult survivors of retinoblastoma (Rb). METHODS The Retinoblastoma Survivor Study is a retrospective cohort of adult survivors of Rb diagnosed between 1932 and 1994. Participants completed a comprehensive questionnaire adapted from the Childhood Cancer Survivor Study surveys. Chronic conditions were classified using the National Cancer Institute's Common Terminology Criteria for Adverse Events (version 4.03). Multivariate Poisson regression was used to compare survivors of Rb with 2377 non-Rb controls, consisting of the Childhood Cancer Survivor Study sibling cohort and survivors with bilateral versus unilateral disease. RESULTS Survivors of Rb (53.6% with bilateral disease) and non-Rb controls had a mean age of 43.3 years (standard deviation, 11 years) and 37.6 years (SD, 8.6 years), respectively, at the time of study enrollment. At a median follow-up of 42 years (range, 15-75 years), 86.6% of survivors of Rb had at least 1 condition and 71.1% had a severe/life-threatening (grade 3-4) condition. The adjusted relative risk (RR) of a chronic condition in survivors compared with non-Rb controls was 1.4 (95% confidence interval [95% CI], 1.3-1.4; P<.01); for a grade 3 to 4 condition, the RR was 7.6 (95% CI, 6.4-8.9; P<.01). Survivors were at an excess risk regardless of laterality. After stratifying by laterality and excluding ocular conditions and second malignant neoplasms (SMNs), only those with bilateral disease were found to be at an increased risk of any nonocular, non-SMN condition (RR, 1.2; 95% CI, 1.1-1.2) and for grade 3 to 4 nonocular, non-SMN conditions (RR, 1.7; 95% CI, 1.2-2.5). CONCLUSIONS Survivors of Rb have an increased risk of chronic conditions compared with non-Rb controls. After excluding ocular conditions and SMNs, this excess risk was found to persist only for those with bilateral disease. © 2016 American Cancer Society.
Keywords: follow-up studies; retinoblastoma; questionnaires; risk; survivors
Journal Title: Cancer
Volume: 122
Issue: 5
ISSN: 0008-543X
Publisher: Wiley Blackwell  
Date Published: 2016-03-01
Start Page: 773
End Page: 781
Language: English
DOI: 10.1002/cncr.29704
PROVIDER: scopus
PMCID: PMC4764449
PUBMED: 26755259
DOI/URL:
Notes: Article -- Export Date: 15 April 2016 -- Source: Scopus
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MSK Authors
  1. Yuelin Li
    136 Li
  2. Joanne Fu-Lou Chou
    145 Chou
  3. Brian Marr
    108 Marr
  4. Ira J Dunkel
    250 Dunkel
  5. Charles A Sklar
    262 Sklar
  6. Jennifer S Ford
    57 Ford
  7. David H Abramson
    247 Abramson
  8. Kevin Oeffinger
    248 Oeffinger
  9. Mary McCabe
    93 McCabe