Increasing Incidence of Neuroblastoma and Potentially Higher Associated Mortality of Children From Nonmetropolitan Areas: Analysis of the Surveillance, Epidemiology, and End Results Database

Scott Department of Urology, Division of Pediatric Urology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX 77030, USA.
Journal of Pediatric Hematology/Oncology (Impact Factor: 0.9). 10/2009; 31(12):942-6. DOI: 10.1097/MPH.0b013e3181bcc809
Source: PubMed


Geographic variations in cancer incidence and mortality can yield clues regarding etiology and healthcare access. We examined pediatric neuroblastoma incidence and mortality in metropolitan counties (containing a core urban area of 50,000 or more people) versus nonmetropolitan counties. We identified cases through the Surveillance, Epidemiology, and End Results database. We analyzed age, race, sex, year of diagnosis, mortality, and county type (metropolitan or nonmetropolitan). In total, 1777 neuroblastoma cases were identified. From 1973 to 2003, the incidence of pediatric neuroblastoma in nonmetropolitan counties has risen from 0.8 to 1.2 cases per 100,000 person-years (annual percent change, 2.09%, P<0.05), whereas the incidence in metropolitan counties remained stable at 1.1 cases per 100,000 person-years over the same interval. The numbers of White children, an at-risk group for neuroblastoma, have increased in nonmetropolitan counties more than in metropolitan counties. Mortality rates were higher in nonmetropolitan versus metropolitan counties [hazard ratio 1.28 (95% confidence interval: 1.02-1.62), log-rank test P=0.0357]. The incidence of pediatric neuroblastoma in nonmetropolitan counties seems to be increasing, possibly due to demographic or environmental factors. Mortality seems to be higher in children from nonmetropolitan versus metropolitan counties. However, this study is limited by sample size. These concerning trends warrant further study through means other than Surveillance, Epidemiology, and End Results.

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    • "Neuroblastoma arises from primitive sympathetic neural cells primarily in the adrenal medulla and also in the paraspinal sympathetic ganglia in the neck, chest, abdomen, or pelvis [1]. Although neuroblastoma currently represents 7% of all childhood malignancies or roughly 1 case per 100,000 children per year, only 1 case per 10 million adults per year is diagnosed in adulthood [2] [3] [4]. Because of the rarity of adult neuroblastoma, staging systems and risk assessment tools have been developed using primarily pediatric data [5]. "
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    ABSTRACT: Background. Staging and treatment of adult neuroblastoma has yet to be formalized. We sought to determine the utility of the pediatric classification system in adults and determine the efficacy of different treatment modalities. Methods. Medical records of 118 adults (patients >17 years old) and 112 pediatric patients (ages 2-17), who were treated for neuroblastoma at M.D. Anderson Cancer Center from January 1994 to September 2012, were reviewed. International neuroblastoma risk group (INRG) variables were abstracted. The primary outcome of interest was actuarial progression-free survival. Results. Median age of pediatric patients was 5 years (range 3-16) and 47 years (range 18-82) for adult patients. There were no differences in PFS or OS between stage-matched risk categories between pediatric and adult patients (L1-P = 0.40, L2-P = 0.54, and M-P = 0.73). In the treatment of L1 disease, median PFS for adults treated with surgery and radiation was 11.1 months compared with single modality local treatment ± chemotherapy (6.4 and 5.1 months, resp.; P = 0.07). Median PFS in L2 adult patients was 5.2 months with local therapy and 4 months with the addition of chemotherapy (P = 0.23). Conclusions. Adult and pediatric patients with neuroblastoma achieve similar survival outcomes. INRG classification should be employed to stratify adult neuroblastoma patients and help select treatment.
    Full-text · Article · Jun 2014 · Sarcoma
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    • "The peripheral neuroblastic tumour group includes NBL, GNBL and GN. NBL is the most common extracranial solid tumour of childhood and the incidence of pediatric neuroblastoma are increasing [17,18]. MYCN gene amplification is a known molecular marker for aggressive progression of NBL [4]. "
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    ABSTRACT: BACKGROUND: Amplification of MYCN oncogene is an established marker indicating aggressive tumor progression of neuroblastoma (NBL). But Copy number analyses of MYCN gene in ganglioneuroblastoma (GNBL) and ganglioneuroma(GN) is poorly described in the literature. In the study, we evaluated the copy number aberrations of MYCN gene in clinical samples of NBLs, GNBLs and GNs and analyzed their association with clinical outcome of the patients. METHODS: In this study, we analyzed MYCN gene and chromosome 2 aneusomy by using fluorescence in situ hybridization (FISH) method in a total of 220 patients with NBL, GNBL and GN cases. Kaplan-Meier curves were generated by using SPSS 12.0 software. RESULTS: Of 220 patients, 178 (81.0%) were NBLs, 32 (14.5%) were GNBLs and 10 (4.5%) were GNs. MYCN gain is a recurrent genetic aberration of neuroblastic tumors (71.8%, 158/220), which was found in 129 NBLs (58.6%, 129/220), 25 GNBLs (11.4%, 25/220) and 4 GN cases (1.8%, 4/220). However, MYCN amplification was only present in 24 NBL tumors (13.5%, 24/178) and 1 GNBL case (3.1%, 1/32). Kaplan-Meier survival analysis indicated that MYCN amplification is significantly correlated with decreased overall survival in NBLs (P=0.017). Furthermore, a better prognosis trend was observed in patients with MYCN gain tumors compared with those with MYCN gene normal copy number tumors and MYCN amplification tumors (P=0.012). CONCLUSIONS: In summary, the frequency of MYCN amplification in NBLs is high and is rarely observed in GNBLs and GNs, which suggest MYCN plays an important role in neuroblastic tumors differentiation. MYCN gain appeared to define a subgroup of NBLs with much better outcome and classification of MYCN gene copy number alteration as three groups (amplification, gain and normal) can provide a powerful prognostic indicator in NBLs. Virtual slides The virtual slide(s) for this article can be found here:
    Full-text · Article · Jan 2013 · Diagnostic Pathology
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    • "NB has the fourth highest incidence rate in children less than 14 years of age in developed countries [15,16]. In a recent analysis of the Surveillance, Epidemiology, and End Results database, the incidence of NB seems to be increasing in nonmetropolitan counties of the United States [17]. A very low incidence rate was observed in Mexico, even in children under one year of age [18,19]. "
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    ABSTRACT: Childhood cancer differs from most common adult cancers, suggesting a distinct aetiology for some types of childhood cancer. Our objective in this study was to test the difference in incidence rates of 4 non-CNS embryonic tumours and their correlation with socioeconomic status (SES) in Brazil. Data was obtained from 13 Brazilian population-based cancer registries (PBCRs) of neuroblastoma (NB), Wilms'tumour (WT), retinoblastoma (RB), and hepatoblastoma (HB). Incidence rates by tumour type, age, and gender were calculated per one million children. Correlations between social exclusion index (SEI) as an indicator of socioeconomic status (SES) and incidence rates was investigated using the Spearman's test. WT, RB, and HB presented with the highest age-adjusted incidence rates (AAIRs) in 1 to 4 year old of both genders, whereas NB presented the highest AAIR in ≤11 month-olds. However, differences in the incidence rates among PBCRs were observed. Higher incidence rates were found for WT and RB, whereas lower incidence rates were observed for NB. Higher SEI was correlated with higher incidences of NB (0.731; p = 0.0117), whereas no SEI correlation was observed between incidence rates for WT, RB, and HB. In two Brazilian cities, the incidence rates of NB and RB were directly correlated with SEI; NB had the highest incidence rates (14.2, 95% CI, 8.6-19.7), and RB the lowest (3.5, 95% CI, 0.7-6.3) in Curitiba (SEI, 0.730). In Natal (SEI, 0.595), we observed just the opposite; the highest incidence rate was for RB and the lowest was for NB (4.6, 95% CI, 0.1-9.1). Regional variations of SES and the incidence of embryonal tumours were observed, particularly incidence rates for NB and RB. Further studies are necessary to investigate risk factors for embryonic tumours in Brazil.
    Full-text · Article · May 2011 · BMC Cancer
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