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Original Investigation
June 2018

Vision-Targeted Health-Related Quality of Life in Adult Survivors of Retinoblastoma

Author Affiliations
  • 1Memorial Sloan Kettering Cancer Center, New York, New York
  • 2Weill Cornell Medical College, New York, New York
  • 3St. Jude Children’s Research Hospital, Memphis, Tennessee
  • 4Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, US Department of Health and Human Services, Bethesda, Maryland
  • 5Duke University, Durham, North Carolina
JAMA Ophthalmol. 2018;136(6):637-641. doi:10.1001/jamaophthalmol.2018.1082
Key Points

Question  How is treatment of retinoblastoma associated with patient-reported vision-focused health-related quality of life during adulthood?

Findings  In this cohort study, oculo-visual problems were associated with patient-reported vision-targeted health-related quality of life in adult retinoblastoma survivors, particularly among those with a history of bilateral disease or enucleation. Despite deficits in a few specific domains, overall patient-reported vision-focused HRQoL was largely preserved in this cohort treated for eye tumors at a very young age.

Meaning  This study supports the need for continued development of vision-preserving and eye-sparing procedures in the treatment of retinoblastoma, when possible, and suggests long-term survivors should be assessed for specific areas of need to strive for optimization of supportive services.


Importance  Retinoblastoma survivors are at risk for adverse oculo-visual outcomes. Limited data are available regarding long-term vision-targeted health-related quality of life (HRQoL) of adult retinoblastoma survivors.

Objective  To examine vision-targeted HRQoL as reported on the 25-item National Eye Institute Visual Field Questionnaire for overall and specific scale scores among adult survivors of retinoblastoma.

Design, Setting, and Participants  The Retinoblastoma Survivor Study is a retrospective cohort of adult retinoblastoma survivors treated at 3 academic medical centers in New York between 1932 and 1994. Participants completed a comprehensive questionnaire between April 2008 and June 2010. Items were scored in January 2013 and preliminary analyses were performed in July 2015. Models were finalized in May 2017.

Main Outcomes and Measures  Self-reported vision-targeted HRQoL as reported on the 25-item National Eye Institute Visual Field Questionnaire. Items are scored from 0 to 100, with 100 representing the highest quality of life.

Results  Among 470 adult retinoblastoma survivors (53.6% with bilateral disease; 52.1% female; 86.4% white and non-Hispanic; mean age at study, 43.3 years; range, 18.0-77.0 years), 86% had at least 1 eye removed (1 eye, 74.5%; both eyes, 11.5%); 56.5% were previously treated with radiotherapy; and 61.3% rated their eyesight as excellent/good while 16.2% reported complete blindness. The overall mean (SD) VFQ composite score for all survivors was 81.1 (17.2) (mean [SD] score for unilateral retinoblastoma survivors, 91.4 [7.7]; bilateral retinoblastoma survivors, 72.3 [18.2]; difference between survivors with unilateral and bilateral disease, 19.1 [95% CI, 16.5-21.7; P < .001]). Prior exposure to radiotherapy was not associated with decreased overall VFQ (β = −0.08; 95% CI, −0.15 to 0.002; P = .06) but was related to a few specific subdomains of visual functioning.

Conclusions and Relevance  These findings suggest retinoblastoma-related oculo-visual problems are associated with functional status and vision-targeted HRQoL of adult survivors, particularly among those with bilateral disease.