Berthold, Frank ORCID: 0000-0002-7613-1723, Ernst, Angela ORCID: 0000-0003-2375-1889, Ackermann, Sandra, Bartenhagen, Christoph, Christiansen, Holger, Hero, Barbara, Rosswog, Carolina, von Schweinitz, Dietrich, Klingebiel, Thomas, Schmid, Irene, Simon, Thorsten ORCID: 0000-0002-3425-8451 and Fischer, Matthias (2021). Genetic Alterations and Resectability Predict Outcome in Patients with Neuroblastoma Assigned to High-Risk Solely by MYCN Amplification. Cancers, 13 (17). BASEL: MDPI. ISSN 2072-6694

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Abstract

Background: To identify variables predicting outcome in neuroblastoma patients assigned to the high-risk group solely by the presence of MYCN oncogene amplification (MNA). Methods: Clinical characteristics, genomic information, and outcome of 190 patients solely assigned to high-risk neuroblastoma by MNA were analyzed and compared to 205 patients with stage 4 neuroblastoma aged >= 18 months with MNA (control group). Results: Event-free survival (EFS) and overall survival (OS) at 10 years were 47% (95%-CI 39-54%) and 56% (95%-CI 49-63%), respectively, which was significantly better than EFS and OS of the control group (EFS 25%, 95%-CI 18-31%, p < 0.001; OS 32% 95%-CI 25-39%, p < 0.001). The presence of RAS-/p53-pathway gene alterations was associated with impaired 10-year EFS and OS (19% vs. 55%, and 19% vs. 67%, respectively; both p < 0.001). In time-dependent multivariable analyses, alterations of RAS-/p53-pathway genes and the extent of the best primary tumor resection were the only independent prognostic variables for OS (p < 0.001 and p = 0.011, respectively). Conclusions: Neuroblastoma patients attributed to high risk solely by MYCN amplification have generally a more favorable outcome. Mutations of genes of the RAS and/or p53 pathways and incomplete resection are the main risk factors predicting poor outcome.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Berthold, FrankUNSPECIFIEDorcid.org/0000-0002-7613-1723UNSPECIFIED
Ernst, AngelaUNSPECIFIEDorcid.org/0000-0003-2375-1889UNSPECIFIED
Ackermann, SandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bartenhagen, ChristophUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Christiansen, HolgerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hero, BarbaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rosswog, CarolinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Schweinitz, DietrichUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klingebiel, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schmid, IreneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Simon, ThorstenUNSPECIFIEDorcid.org/0000-0002-3425-8451UNSPECIFIED
Fischer, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-590359
DOI: 10.3390/cancers13174360
Journal or Publication Title: Cancers
Volume: 13
Number: 17
Date: 2021
Publisher: MDPI
Place of Publication: BASEL
ISSN: 2072-6694
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
STAGE-3 NEUROBLASTOMA; OPEN-LABEL; SURVIVAL; TUMORS; CLASSIFICATION; CHEMOTHERAPY; PATHOLOGY; RESECTION; CHILDREN; CRITERIAMultiple languages
OncologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/59035

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