title: Developmental and adaptive functioning in children with retinoblastoma: a longitudinal investigation.
abstract: OBJECTIVE
  To determine the developmental trajectory of early cognitive and adaptive skills in young children with retinoblastoma from diagnosis to 5 years of age.
  
  
  METHODS
  Ninety-four patients with retinoblastoma treated according to an institutional protocol underwent serial assessments of cognitive and adaptive functioning at age 6 months and 1, 2, 3, and 5 years. Data were analyzed by treatment strata, with patients with 13q deletion analyzed separately.
  
  
  RESULTS
  At baseline, across all patients (except those with 13q deletion), developmental functioning was comparable with the normative mean, with mean scores for all strata within the average range. However, at age 5 years, developmental functioning was in the low average range and significantly below normative means. The trajectories of developmental functioning demonstrated significant decline over time, although this varied by treatment group/strata. Patients treated with enucleation only evidenced the greatest decline in cognitive functioning; significant change was not observed in patients treated with other modalities. Notable declines in parent-reported communication skills were observed in the majority of patients. Patients with 13q deletion evidenced delayed cognitive functioning at baseline, but minimal declines were observed through age 3 years. However, significant decreases in adaptive functioning were demonstrated over time for the 13q deletion subset.
  
  
  CONCLUSIONS
  The declines in functioning observed in this study were unexpected, as was the poorer performance of the enucleation-only group. This highlights the necessity of continuing to assess cognitive functioning in patients with retinoblastoma as they age. Additional research is necessary to determine the long-term trajectory of cognitive development in this population.
authors.affiliation: Victoria W. Willard, Ibrahim Qaddoumi, Si Chen, Hui Zhang, Rachel Brennan, and Sean Phipps, St Jude Children's Research Hospital; Matthew W. Wilson, University of Tennessee, Memphis, TN; and Carlos Rodriguez-Galindo, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA.
authors.forename: Victoria W
authors.initials: VW
authors.lastname: Willard
authors.affiliation: Victoria W. Willard, Ibrahim Qaddoumi, Si Chen, Hui Zhang, Rachel Brennan, and Sean Phipps, St Jude Children's Research Hospital; Matthew W. Wilson, University of Tennessee, Memphis, TN; and Carlos Rodriguez-Galindo, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA.
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Qaddoumi
authors.affiliation: Victoria W. Willard, Ibrahim Qaddoumi, Si Chen, Hui Zhang, Rachel Brennan, and Sean Phipps, St Jude Children's Research Hospital; Matthew W. Wilson, University of Tennessee, Memphis, TN; and Carlos Rodriguez-Galindo, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA.
authors.forename: Si
authors.initials: S
authors.lastname: Chen
authors.affiliation: Victoria W. Willard, Ibrahim Qaddoumi, Si Chen, Hui Zhang, Rachel Brennan, and Sean Phipps, St Jude Children's Research Hospital; Matthew W. Wilson, University of Tennessee, Memphis, TN; and Carlos Rodriguez-Galindo, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA.
authors.forename: Hui
authors.initials: H
authors.lastname: Zhang
authors.affiliation: Victoria W. Willard, Ibrahim Qaddoumi, Si Chen, Hui Zhang, Rachel Brennan, and Sean Phipps, St Jude Children's Research Hospital; Matthew W. Wilson, University of Tennessee, Memphis, TN; and Carlos Rodriguez-Galindo, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA.
authors.forename: Rachel
authors.initials: R
authors.lastname: Brennan
authors.affiliation: Victoria W. Willard, Ibrahim Qaddoumi, Si Chen, Hui Zhang, Rachel Brennan, and Sean Phipps, St Jude Children's Research Hospital; Matthew W. Wilson, University of Tennessee, Memphis, TN; and Carlos Rodriguez-Galindo, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA.
authors.forename: Carlos
authors.initials: C
authors.lastname: Rodriguez-Galindo
authors.affiliation: Victoria W. Willard, Ibrahim Qaddoumi, Si Chen, Hui Zhang, Rachel Brennan, and Sean Phipps, St Jude Children's Research Hospital; Matthew W. Wilson, University of Tennessee, Memphis, TN; and Carlos Rodriguez-Galindo, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA.
authors.forename: Matthew W
authors.initials: MW
authors.lastname: Wilson
authors.affiliation: Victoria W. Willard, Ibrahim Qaddoumi, Si Chen, Hui Zhang, Rachel Brennan, and Sean Phipps, St Jude Children's Research Hospital; Matthew W. Wilson, University of Tennessee, Memphis, TN; and Carlos Rodriguez-Galindo, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA. sean.phipps@stjude.org.
authors.forename: Sean
authors.initials: S
authors.lastname: Phipps
mesh_terms: D000222:Adaptation, Physiological / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D003071:Cognition / Q000502:physiology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008297:Male; D019572:Retinal Neoplasms / Q000503:physiopathology* / Q000523:psychology; D012175:Retinoblastoma / Q000503:physiopathology* / Q000523:psychology