UCLA

Retinoblastoma (RB) is the most common malignant intraocular tumor occurring in childhood. While survival rates in the United States (U.S.) are high, children are left with the impact of treatment including enucleation, visual impairment or blindness as well as an increased risk of secondary cancer in those with the heritable form. Little is known about the influence of these factors on the health-related quality of life (HRQOL) in RB survivors. The first manuscript describes an extensive review of the literature on HRQOL in the adolescent and young adult (AYA) RB population. The findings support the need for HRQOL and vision-related quality of life (VRQOL) research in RB survivors. The second manuscript is a comparative, cross-sectional study of 101 AYA (aged 14-26 years) RB survivors and 97 age-, gender- and race/ethnicity-matched healthy controls. Measures included the Patient Reported Outcomes Measurement Information System PROMIS�-29 Profile, the National Institute of Health (NIH) Toolbox� VRQOL Survey, the Rosenberg Self-Esteem Scale and the Multidimensional Scale of Perceived Social Support. RB survivors reported worse physical functioning than controls but PROMIS-29 physical and mental health summary scores were comparable. Social support and self-esteem were worse in the RB group with self-esteem significantly associated with better mental and physical HRQOL. Visual acuity and self-esteem accounted for 52% of the variance in the PROMIS-29 physical health summary score. The third manuscript examines unilateral verses bilateral RB survivors which identified bilateral having worse VRQOL than unilateral survivors on all domains except ocular symptoms and psychosocial well-being. Worse scores were reported on the VRQOL psychosocial and role performance domains than other domains of the VRQOL. The VRQOL domains had medium to large correlations with the majority of PROMIS-29 scales and summary scores. A single-item measure of overall QOL had medium correlations with the VRQOL psychosocial and role performance scales. The study findings contribute to gaps in our understanding of HRQOL in RB survivors. RB survivors had deficits in physical function and self-esteem with self-esteem and visual acuity as independent predictors of HRQOL. The NIH Toolbox� VRQOL provides more detailed insight into specific vision related effects on daily life of AYA RB.