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      Association of MYCN copy number with clinical features, tumor biology, and outcomes in neuroblastoma: A report from the Children's Oncology Group : MYCN Copy Number in Neuroblastoma

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          Abstract

          High-level MYCN amplification (MNA) is associated with poor outcome and unfavorable clinical and biological features in patients with neuroblastoma. To the authors' knowledge, less is known regarding these associations in patients with low-level MYCN copy number increases.

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          Most cited references27

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          The International Neuroblastoma Risk Group (INRG) classification system: an INRG Task Force report.

          Because current approaches to risk classification and treatment stratification for children with neuroblastoma (NB) vary greatly throughout the world, it is difficult to directly compare risk-based clinical trials. The International Neuroblastoma Risk Group (INRG) classification system was developed to establish a consensus approach for pretreatment risk stratification. The statistical and clinical significance of 13 potential prognostic factors were analyzed in a cohort of 8,800 children diagnosed with NB between 1990 and 2002 from North America and Australia (Children's Oncology Group), Europe (International Society of Pediatric Oncology Europe Neuroblastoma Group and German Pediatric Oncology and Hematology Group), and Japan. Survival tree regression analyses using event-free survival (EFS) as the primary end point were performed to test the prognostic significance of the 13 factors. Stage, age, histologic category, grade of tumor differentiation, the status of the MYCN oncogene, chromosome 11q status, and DNA ploidy were the most highly statistically significant and clinically relevant factors. A new staging system (INRG Staging System) based on clinical criteria and tumor imaging was developed for the INRG Classification System. The optimal age cutoff was determined to be between 15 and 19 months, and 18 months was selected for the classification system. Sixteen pretreatment groups were defined on the basis of clinical criteria and statistically significantly different EFS of the cohort stratified by the INRG criteria. Patients with 5-year EFS more than 85%, more than 75% to or = 50% to < or = 75%, or less than 50% were classified as very low risk, low risk, intermediate risk, or high risk, respectively. By defining homogenous pretreatment patient cohorts, the INRG classification system will greatly facilitate the comparison of risk-based clinical trials conducted in different regions of the world and the development of international collaborative studies.
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            Tests for Linear Trends in Proportions and Frequencies

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              Association of multiple copies of the N-myc oncogene with rapid progression of neuroblastomas.

              Eighty-nine patients with untreated primary neuroblastomas were studied to determine the relation between the number of copies of the N-myc oncogene and survival without disease progression. Genomic amplification (3 to 300 copies) of N-myc was detected in 2 of 16 tumors in Stage II, 13 of 20 in Stage III, and 19 of 40 in Stage IV; in contrast, 8 Stage I and 5 Stage IV-S tumors all had 1 copy of the gene (P less than 0.01). Analysis of progression-free survival in all patients revealed that amplification of N-myc was associated with the worst prognosis (P less than 0.0001); the estimated progression-free survival at 18 months was 70 per cent, 30 per cent, and 5 per cent for patients whose tumors had 1, 3 to 10, or more than 10 N-myc copies, respectively. Of 16 Stage II tumors, 2 with amplification metastasized, whereas only 1 of 14 without amplification did so (P = 0.03). Stage IV tumors with amplification progressed most rapidly: nine months after diagnosis the estimated progression-free survival was 61 per cent, 47 per cent, and 0 per cent in patients whose tumors had 1, 3 to 10, or more than 10 copies, respectively (P less than 0.0001). These results suggest that genomic amplification of N-myc may have a key role in determining the aggressiveness of neuroblastomas.
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                Author and article information

                Journal
                Cancer
                Cancer
                Wiley
                0008543X
                November 01 2017
                November 01 2017
                July 11 2017
                : 123
                : 21
                : 4224-4235
                Affiliations
                [1 ]Dana-Farber/Boston Children's Cancer and Blood Disorders Center; Harvard Medical School; Boston Massachusetts
                [2 ]Institute for Genomic Medicine; Nationwide Children's Hospital; Columbus Ohio
                [3 ]Department of Pathology; The Ohio State University College of Medicine; Columbus Ohio
                [4 ]Department of Pediatrics; The Ohio State University College of Medicine; Columbus Ohio
                [5 ]Children's Oncology Group Statistics and Data Center; University of Florida; Gainesville Florida
                [6 ]Department of Pediatrics; Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania; Philadelphia Pennsylvania
                [7 ]Department of Pediatrics; University of California at San Francisco Benioff Children's Hospital, University of California at San Francisco School of Medicine; San Francisco California
                [8 ]Department of Pediatrics; The Hospital for Sick Children; Toronto Ontario Canada
                [9 ]Department of Pathology; Children's Hospital of Los Angeles; Los Angeles California
                [10 ]Department of Hematology/Oncology; Cook Children's Hospital; Fort Worth Texas
                [11 ]Department of Pediatrics; Seattle Children's Hospital, University of Washington; Seattle Washington
                Article
                10.1002/cncr.30873
                5650521
                28696504
                653a53aa-33c4-4ee9-95a5-3980f0364080
                © 2017

                http://doi.wiley.com/10.1002/tdm_license_1.1

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