Long-term outcomes in survivors of neuroblastoma: A report from the childhood cancer survivor study Journal Article


Authors: Laverdiere, C.; Liu, Q.; Yasui, Y.; Nathan, P. C.; Gurney, J. G.; Stovall, M.; Diller, L. R.; Cheung, N. K.; Wolden, S.; Robison, L. L.; Sklar, C. A.
Article Title: Long-term outcomes in survivors of neuroblastoma: A report from the childhood cancer survivor study
Abstract: 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.MethodsOutcome 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.ResultsSix 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).ConclusionsNeuroblastoma 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.
Keywords: controlled study; treatment outcome; survival rate; acute granulocytic leukemia; major clinical study; cisplatin; united states; outcome assessment; brain tumor; cancer diagnosis; cancer incidence; demography; quality of life; neoplasm recurrence, local; breast cancer; etoposide; incidence; cohort analysis; odds ratio; alkylating agent; cyclophosphamide; risk factor; kidney carcinoma; cancer mortality; growth hormone; acute lymphoblastic leukemia; childhood cancer; growth hormone deficiency; hodgkin disease; ovary insufficiency; cancer survivor; risk assessment; siblings; survivors; chronic disease; survival time; health status; neuroblastoma; soft tissue sarcoma; recurrent disease; cataract; sex difference; thyroid cancer; cancer epidemiology; age distribution; neoplasms, second primary; second cancer; hypothyroidism; sibling; employment status; marriage; social aspect; neurological complication; canada; blindness; musculoskeletal disease; hearing loss; drug exposure; income
Journal Title: JNCI: Journal of the National Cancer Institute
Volume: 101
Issue: 16
ISSN: 0027-8874
Publisher: Oxford University Press  
Date Published: 2009-08-01
Start Page: 1131
End Page: 1140
Language: English
DOI: 10.1093/jnci/djp230
PUBMED: 19648511
PROVIDER: scopus
PMCID: PMC2728747
DOI/URL:
Notes: --- - "Cited By (since 1996): 6" - "Export Date: 30 November 2010" - "CODEN: JNCIA" - "Source: Scopus"
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MSK Authors
  1. Nai-Kong Cheung
    532 Cheung
  2. Charles A Sklar
    299 Sklar
  3. Suzanne L Wolden
    456 Wolden