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. Cancer 2016;122:773-781. © 2016 American Cancer Society.
authors.affiliation: Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Danielle Novetsky
authors.initials: DN
authors.lastname: Friedman
authors.affiliation: Department of Epidemiology-Biostatistics, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Joanne F
authors.initials: JF
authors.lastname: Chou
authors.affiliation: Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, New York.|Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Kevin C
authors.initials: KC
authors.lastname: Oeffinger
authors.affiliation: Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Rockville, Maryland.
authors.forename: Ruth A
authors.initials: RA
authors.lastname: Kleinerman
authors.affiliation: Department of Psychiatry and Behavioral Sciences, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Jennifer S
authors.initials: JS
authors.lastname: Ford
authors.affiliation: Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Charles A
authors.initials: CA
authors.lastname: Sklar
authors.affiliation: Department of Epidemiology-Biostatistics, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Yuelin
authors.initials: Y
authors.lastname: Li
authors.affiliation: Clinical Director, Survivorship Center, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Mary S
authors.initials: MS
authors.lastname: McCabe
authors.affiliation: Department of Epidemiology and Cancer Control, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Leslie L
authors.initials: LL
authors.lastname: Robison
authors.affiliation: Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Brian P
authors.initials: BP
authors.lastname: Marr
authors.affiliation: Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: David H
authors.initials: DH
authors.lastname: Abramson
authors.affiliation: Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Ira J
authors.initials: IJ
authors.lastname: Dunkel
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D002386:Cataract / Q000453:epidemiology*; D002908:Chronic Disease; D015331:Cohort Studies; D015897:Comorbidity; D003920:Diabetes Mellitus / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D034381:Hearing Loss / Q000453:epidemiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016609:Neoplasms, Second Primary / Q000453:epidemiology*; D012044:Regression Analysis; D019572:Retinal Neoplasms / Q000453:epidemiology* / Q000628:therapy; D012175:Retinoblastoma / Q000453:epidemiology* / Q000628:therapy; D012189:Retrospective Studies; D020521:Stroke / Q000453:epidemiology; D017741:Survivors / Q000706:statistics & numerical data*; D016606:Thyroid Nodule / Q000453:epidemiology*; D014786:Vision Disorders / Q000453:epidemiology*
keywords: follow-up studies; questionnaires; retinoblastoma; risk; survivors