Background
Limited data are available on long-term morbidity in adult retinoblastoma (Rb) survivors.
Methods
The Rb Survivor Study is a retrospective cohort of adult Rb survivors diagnosed between 1932 and 1994. Participants completed a comprehensive questionnaire, adapted from the Childhood Cancer Survivor Study (CCSS) surveys. Chronic conditions were classified using the Common Terminology Criteria for Adverse Events v4.03. Multivariate Poisson regression was used to compare Rb survivors with 2,377 non-Rb controls, consisting of the CCSS sibling cohort, and survivors with bilateral versus unilateral disease.
Results
Rb survivors (53.6% with bilateral disease) and non-Rb controls had mean ages of 43.3 (SD 11) and 37.6 (SD 8.6) years, respectively, at study enrollment. At a median follow-up of 42 years (range, 15-75), 86.6% of Rb survivors had at least one condition and 71.1% had a severe/life-threatening (grade 3-4) condition. The adjusted relative risk of a chronic condition in survivors, compared to non-Rb controls, was 1.4 (95% confidence interval [CI], 1.3–1.4, p<0.01); for a grade 3-4 condition, the risk was 7.6 (95% CI, 6.4–8.9, p<0.01). Survivors were at excess risk regardless of laterality. After stratifying by laterality and excluding ocular conditions and SMN, only those with bilateral disease were at increased risk for any non-ocular, non-SMN condition (RR 1.2; 95% CI, 1.1–1.2) and for grade 3-4 non-ocular, non-SMN conditions (RR 1.7; 95% CI, 1.2–2.5).
Conclusions
Rb survivors have an increased risk of chronic conditions compared to non-Rb controls. After excluding ocular conditions and SMN, this excess risk persists only for those with bilateral disease.