Cognitive function and social attainment in adult survivors of retinoblastoma: a report from the St. Jude Lifetime Cohort Study.

BACKGROUND: Retinoblastoma has a 5-year survival rate exceeding 95%, yet little is known about long-term functional outcomes for these patients. METHODS: Sixty-nine adult survivors of retinoblastoma (mean age, 33 years; mean years post-diagnosis, 31) who had enrolled in the St. Jude Lifetime Cohort Study completed clinical cognitive evaluations and questionnaires assessing adult social attainment. Scores on all cognitive measures were converted to z-scores (M = 0, SD = 1) using age-adjusted normative data. Multivariable linear regression analyses, adjusted for age at diagnosis and disease laterality, were used to examine associations between disease and treatment exposures and cognitive outcomes. RESULTS: Retinoblastoma survivors performed within normative expectations across most cognitive domains. In multivariable models, adjusted for disease laterality, survivors diagnosed at ≤1 year of age performed significantly better on measures of short-term verbal memory (ß = 0.87, P<.01), long-term verbal memory (ß = 0.66, P = .02), verbal learning (ß = 0.67, P = .02), and verbal reasoning abilities (ß = 0.79, P<.01) compared with survivors diagnosed at >1 year of age. In multivariable models, restricted to bilateral survivors and adjusted for age at diagnosis, whole brain radiation exposure was significantly associated with poorer performance on tasks of short-term verbal memory (ß = -0.003, P = .03) and long-term verbal memory (ß = -0.003, P = .01). Reported social attainment was consistent with adult developmental expectations. CONCLUSIONS: Adult survivors of retinoblastoma demonstrate few cognitive or social attainment deficits decades following diagnosis and treatment. Findings suggest the potential for neural reorganization following early insult to the visual system as well as vulnerability of the developing brain to low dose radiation exposure. Early intervention and rehabilitation will be important for these patients.

Social attainment in survivors of pediatric central nervous system tumors: a systematic review and meta-analysis from the Children's Oncology Group.

PURPOSE: Improved therapies for pediatric central nervous system (CNS) tumors have increased survival rates; however, many survivors experience significant long-term functional limitations. Survivors of pediatric CNS tumors can experience deficits in social attainment. The aim of this review was to systematically amalgamate findings pertaining to social attainment (i.e., educational attainment, marriage, employment outcomes) in survivors of pediatric CNS tumors. METHODS: PubMed (web-based), PsycINFO (EBSCO), EMBASE (Ovid), and Web of Science (Thomson Reuters) were used to identify articles published between January 2011 and September 2018. Eligible studies reported outcomes for survivors of pediatric CNS tumors diagnosed before age 21 years and > 5 years from diagnosis and/or > 2 years off therapy. All data were independently abstracted by two reviewers. Random-effects meta-analyses were performed using Review Manager 5.0. RESULTS: The search yielded 7021 unique publications. Forty-six were included in the current review. Meta-analyses revealed survivors of CNS tumors were significantly more likely to have completed compulsory education only (OR = 1.87, 95% CI = 1.66, 2.12, p < 0.00001), less likely to be married (OR = 4.70, 95% CI = 3.89, 5.68, p < 0.00001), and more likely to be unemployed (OR = 2.84, 95% CI = 2.62, 3.08, p < 0.00001) compared to non-cancer controls. Cranial radiation therapy, neurocognitive deficits, and younger age at diagnosis were associated with poorer outcomes. Hearing loss and bilateral blindness were also related to poorer outcomes. Sex did not impact social attainment outcomes. CONCLUSIONS: Survivors of pediatric CNS tumors are at elevated risk for poor attainment of key adult social outcomes. IMPLICATIONS FOR CANCER SURVIVORS: There is a critical need to develop interventions to support survivors in becoming independent and productive adults.

Neurocognitive outcomes in long-term survivors of Wilms tumor: a report from the St. Jude Lifetime Cohort.

PURPOSE: To examine prevalence and predictors of neurocognitive outcomes, social attainment, emotional distress, and health-related quality of life (HRQOL) in long-term survivors of pediatric Wilms tumor (WT). METHODS: One hundred fifty-eight WT survivors (59% female; mean [SD] age 33 [9.1] years; time since diagnosis 29 [9.1] years) and 354 community controls (55.6% female; 35 [10.2] years) completed comprehensive neuropsychological testing and physical examination, including echocardiography/electrocardiography, pulmonary function tests, and endocrine evaluation. Self-report of emotional distress, HRQOL, and social attainment were collected. Impairment was defined in relation to both controls and normative data. Generalized linear models were developed to examine impact of treatment and chronic health conditions on outcomes. RESULTS: WT survivors performed poorer than norms and controls in 6 of 16 cognitive variables and 1 of 8 HRQOL variables, with scores ranging from - 0.64 (mathematics) to - 0.21 (verbal fluency) standard deviations below expectations. Compared to controls, WT survivors were less likely to graduate college (odds ratio 2.23, 95% confidence interval 1.46-3.41) and had more moderate to severe neurologic conditions (18.4% vs 8.2%, p < 0.001), which were associated with poor memory (ß = - 0.90, p < 0.001), attention (ß = - 1.02, p < 0.001), and HRQOL general health (ß = - 0.80, p = 0.0015). Treatment variables and cardiopulmonary morbidity (higher in survivors) were not associated with outcomes. CONCLUSIONS: Survivors of WT demonstrate impairment in neurocognitive function and have lower social attainment during adulthood, with poorer neurocognitive function associated with neurologic morbidity. IMPLICATIONS FOR CANCER SURVIVORS: Survivors of WT should be offered neurocognitive evaluations and rehabilitation. Neurologic conditions should be routinely assessed, and appropriate support offered to reduce risk for functional limitations.