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Cancer. 2016 Mar 1;122(5):773-81. doi: 10.1002/cncr.29704. Epub 2016 Jan 11.

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

Author information

  • 1Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, New York.
  • 2Department of Epidemiology-Biostatistics, Memorial Sloan Kettering Cancer Center, New York, New York.
  • 3Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
  • 4Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Rockville, Maryland.
  • 5Department of Psychiatry and Behavioral Sciences, Memorial Sloan Kettering Cancer Center, New York, New York.
  • 6Clinical Director, Survivorship Center, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
  • 7Department of Epidemiology and Cancer Control, St. Jude Children's Research Hospital, Memphis, Tennessee.
  • 8Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, New York, New York.

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. Cancer 2016;122:773-781. © 2016 American Cancer Society.

KEYWORDS:

follow-up studies; questionnaires; retinoblastoma; risk; survivors

PMID:
26755259
PMCID:
PMC4764449
[Available on 2017-03-01]
DOI:
10.1002/cncr.29704
[PubMed - indexed for MEDLINE]
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