Journal of the National Cancer Institute Advance Access originally published online on July 31, 2009
JNCI Journal of the National Cancer Institute 2009 101(16):1131-1140; doi:10.1093/jnci/djp230
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© The Author 2009. Published by Oxford University Press.
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Long-term Outcomes in Survivors of Neuroblastoma: A Report From the Childhood Cancer Survivor Study
Affiliations of authors: Department of Pediatrics, University of Montreal, Montreal, QC, Canada (CL); Department of Public Health Sciences, School of Public Health, University of Alberta, Edmonton, AB, Canada (QL, YY); Department of Pediatrics, Hospital for Sick Children, Toronto, ON, Canada (PCN); Department of Pediatrics, Child Health Evaluation and Research Unit, University of Michigan, Ann Arbor, MI (JGG); Department of Radiation Physics, University of Texas M. D. Anderson Cancer Center, Houston, TX (MS); Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA (LRD); Department of Pediatrics (N-KC, CAS) and Department of Radiation Oncology (SW), Memorial Sloan-Kettering Cancer Center, New York, NY; Department of Epidemiology and Cancer Control, St. Jude Children's Research Hospital, Memphis, TN (LLR)
Correspondence to: Caroline Laverdière, MD, Sainte-Justine Hospital, 3175 Cote Sainte-Catherine, Montreal, QC, Canada H3T 1C5 (e-mail: caroline.laverdiere{at}umontreal.ca).
Background: The 5-year survival rate for individuals with neuroblastoma is approaching 70%. Few data exist, however, on the long-term outcomes of these patients, who are often treated at a very young age.
Methods: Outcome data were obtained for 954 5-year neuroblastoma survivors who were diagnosed in 1970–1986 and enrolled in the Childhood Cancer Survivor Study (CCSS). Late mortality, second malignant neoplasms, and chronic health conditions were analyzed in relation to treatment factors using Poisson regression models and their modification with generalized estimating equations. Neuroblastoma survivors were compared with a cohort of 3899 siblings of CCSS participants for risk of chronic health conditions and selected sociodemographic outcomes. All statistical tests were two-sided.
Results: Six percent of patients died more than 5 years after their diagnosis (standardized mortality ratio = 5.6; 95% confidence interval [CI] = 4.4 to 6.9). The most common causes of death were disease recurrence (n = 43) and second malignant neoplasms (n = 13). The cumulative incidence of second malignant neoplasms was 3.5% at 25 years and 7.0% at 30 years after diagnosis. Compared with the sibling cohort, survivors had an increased risk of selected chronic health conditions (risk ratio [RR] = 8.3; 95% CI = 7.1 to 9.7) with a 20-year cumulative incidence of 41.1%. The most prevalent outcomes involved the neurological, sensory, endocrine, and musculoskeletal systems, with 20-year cumulative incidences of 29.8%, 8.6%, 8.3%, and 7.8%, respectively. Neuroblastoma survivors who were treated with multimodality therapy were more likely to develop a chronic health condition than survivors treated with surgery alone (RR = 2.2; 95% CI = 1.6 to 3.0). Neuroblastoma survivors were less likely than siblings to have ever been employed (P = .04) or to be married (P < .001) and had a lower personal income (P = .009).
Conclusions: Neuroblastoma survivors have an increased rate of mortality and second malignant neoplasms, relative to the age- and sex-comparable US population, and of chronic health conditions, relative to their siblings, which underscores the need for long-term medical surveillance.
| CONTEXT AND CAVEATS Prior knowledge Very limited information has been available about long-term outcomes among survivors of childhood cancers including neuroblastoma. The Childhood Cancer Survivor Study (CCSS) was initiated to study long-term outcomes among those diagnosed in the United States and Canada in 1970–1986. Study design Late mortality was assessed among 1358 5-year survivors of neuroblastoma who were CCSS participants. Of these, 954 participating survivors self-reported demographic information, second malignant neoplasms, and chronic health conditions, from which cumulative incidences could be calculated. Data from 3899 nondiseased siblings were used as a control. Contribution Nearly 6% of patients died more than 5 years after diagnosis, usually from disease recurrence or second malignant neoplasms. Neuroblastoma survivors were eight times more likely than the sibling cohort to have chronic health conditions, and by 20 years, nearly a third would develop neurological complications and about 8% each would develop endocrine, sensory, and musculoskeletal complications. Those who received multimodality therapy were more than twice as likely to develop a chronic health condition as those treated by surgery alone. Implications Emerging therapies should be evaluated for their potential long-term effects. Limitations The data gathered were for participants only, demographic and medical outcomes among survivors were self-reported, and data concerning neuroblastoma stage at diagnosis were unavailable. From the Editors
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Manuscript received October 8, 2008; revised May 8, 2009; accepted June 19, 2009.
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