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 (β = 0.87, P<.01), long-term verbal memory (β = 0.66, P = .02), verbal learning (β = 0.67, P = .02), and verbal reasoning abilities (β = 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 (β = -0.003, P = .03) and long-term verbal memory (β = -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.
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 (β = 0.87, P<.01), long-term verbal memory (β = 0.66, P = .02), verbal learning (β = 0.67, P = .02), and verbal reasoning abilities (β = 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 (β = -0.003, P = .03) and long-term verbal memory (β = -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.
Authors: Brenda J Spiegler; Kimberly Kennedy; Ronnen Maze; Mark L Greenberg; Sheila Weitzman; Johann K Hitzler; Paul C Nathan Journal: J Clin Oncol Date: 2006-08-20 Impact factor: 44.544
Authors: Scott M Barb; Carlos Rodriguez-Galindo; Matthew W Wilson; Nicholas S Phillips; Ping Zou; Matthew A Scoggins; Yimei Li; Ibrahim Qaddoumi; Kathleen J Helton; George Bikhazi; Barrett G Haik; Robert J Ogg Journal: Invest Ophthalmol Vis Sci Date: 2011-04-20 Impact factor: 4.799
Authors: Gregory T Armstrong; Wilburn E Reddick; Ronald C Petersen; Aimee Santucci; Nan Zhang; Deokumar Srivastava; Robert J Ogg; Claudia M Hillenbrand; Noah Sabin; Matthew J Krasin; Larry Kun; Ching-Hon Pui; Melissa M Hudson; Leslie L Robison; Kevin R Krull Journal: J Natl Cancer Inst Date: 2013-04-12 Impact factor: 13.506
Authors: Christopher Janson; Wendy Leisenring; Cheryl Cox; Amanda M Termuhlen; Ann C Mertens; John A Whitton; Pamela Goodman; Lonnie Zeltzer; Leslie L Robison; Kevin R Krull; Nina S Kadan-Lottick Journal: Cancer Epidemiol Biomarkers Prev Date: 2009-10 Impact factor: 4.254
Authors: Peter M K de Blank; Michael J Fisher; Lu Lu; Wendy M Leisenring; Kirsten K Ness; Charles A Sklar; Marilyn Stovall; Chris Vukadinovich; Leslie L Robison; Gregory T Armstrong; Kevin R Krull Journal: Cancer Date: 2016-01-11 Impact factor: 6.860
Authors: Victoria W Willard; James L Klosky; Chenghong Li; Deo Kumar Srivastava; Tara M Brinkman; Leslie L Robison; Melissa M Hudson; Sean Phipps Journal: Cancer Date: 2017-01-18 Impact factor: 6.860
Authors: Helen Dimaras; Timothy W Corson; David Cobrinik; Abby White; Junyang Zhao; Francis L Munier; David H Abramson; Carol L Shields; Guillermo L Chantada; Festus Njuguna; Brenda L Gallie Journal: Nat Rev Dis Primers Date: 2015-08-27 Impact factor: 52.329
Authors: Danielle Novetsky Friedman; Joanne F Chou; Jasmine H Francis; Charles A Sklar; Yuelin Li; Mary McCabe; Leslie L Robison; Ruth A Kleinerman; Kevin C Oeffinger; David H Abramson; Ira J Dunkel; Jennifer S Ford Journal: JAMA Ophthalmol Date: 2018-06-01 Impact factor: 7.389
Authors: Victoria W Willard; Ibrahim Qaddoumi; Haitao Pan; Chia-Wei Hsu; Rachel C Brennan; Matthew W Wilson; Carlos Rodriguez-Galindo; Kristin Goode; Kendra Parris; Sean Phipps Journal: J Clin Oncol Date: 2021-04-23 Impact factor: 50.717
Authors: Kent W Mouw; Beow Y Yeap; Paul Caruso; Aaron Fay; Madhusmita Misra; Roshan V Sethi; Shannon M MacDonald; Yen-Lin Chen; Nancy J Tarbell; Torunn I Yock; Suzanne K Freitag; John E Munzenrider; Eric Grabowski; Michelle Katz; Karen Kuhlthau; Dawn DeCastro; Gena Heidary; Jessica Ciralsky; Shizuo Mukai; Helen A Shih Journal: Adv Radiat Oncol Date: 2017 Jan-Mar
Authors: Sruti S Akella; Jasmine H Francis; Andrea Knezevic; Irina Ostrovnaya; Y Pierre Gobin; Danielle Friedman; Edith Guarini; Lindsey Eibeler; Federica Catalanotti; David H Abramson Journal: PLoS One Date: 2018-05-07 Impact factor: 3.240