Universität zu Köln

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:	Creators Email ORCID ORCID Put Code Berthold, Frank UNSPECIFIED orcid.org/0000-0002-7613-1723 UNSPECIFIED Ernst, Angela UNSPECIFIED orcid.org/0000-0003-2375-1889 UNSPECIFIED Ackermann, Sandra UNSPECIFIED UNSPECIFIED UNSPECIFIED Bartenhagen, Christoph UNSPECIFIED UNSPECIFIED UNSPECIFIED Christiansen, Holger UNSPECIFIED UNSPECIFIED UNSPECIFIED Hero, Barbara UNSPECIFIED UNSPECIFIED UNSPECIFIED Rosswog, Carolina UNSPECIFIED UNSPECIFIED UNSPECIFIED von Schweinitz, Dietrich UNSPECIFIED UNSPECIFIED UNSPECIFIED Klingebiel, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmid, Irene UNSPECIFIED UNSPECIFIED UNSPECIFIED Simon, Thorsten UNSPECIFIED orcid.org/0000-0002-3425-8451 UNSPECIFIED Fischer, Matthias UNSPECIFIED UNSPECIFIED UNSPECIFIED
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:	Keywords Language STAGE-3 NEUROBLASTOMA; OPEN-LABEL; SURVIVAL; TUMORS; CLASSIFICATION; CHEMOTHERAPY; PATHOLOGY; RESECTION; CHILDREN; CRITERIA Multiple languages Oncology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/59035