Frank Berthold, Angela Ernst, Sandra Ackermann, Christoph Bartenhagen, Holger Christiansen, Barbara Hero, Carolina Rosswog, Dietrich von Schweinitz, Thomas Klingebiel, Irene Schmid, Thorsten Simon, Fischer Matthias
- 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.
MetadatenAuthor: | Frank Berthold, Angela Ernst, Sandra Ackermann, Christoph Bartenhagen, Holger Christiansen, Barbara Hero, Carolina Rosswog, Dietrich von Schweinitz, Thomas KlingebielORCiDGND, Irene Schmid, Thorsten Simon, Fischer Matthias |
---|
URN: | urn:nbn:de:hebis:30:3-621744 |
---|
DOI: | https://doi.org/10.3390/cancers13174360 |
---|
ISSN: | 2072-6694 |
---|
Parent Title (English): | Cancers |
---|
Publisher: | MDPI |
---|
Place of publication: | Basel |
---|
Document Type: | Article |
---|
Language: | English |
---|
Date of Publication (online): | 2021/08/28 |
---|
Date of first Publication: | 2021/08/28 |
---|
Publishing Institution: | Universitätsbibliothek Johann Christian Senckenberg |
---|
Release Date: | 2022/03/02 |
---|
Tag: | ALK; MYCN amplification; RAS pathway; high-risk neuroblastoma; p53 pathway; resectability |
---|
Volume: | 13 |
---|
Issue: | 17, art. 4360 |
---|
Page Number: | 15 |
---|
First Page: | 1 |
---|
Last Page: | 15 |
---|
Note: | This research received external funding. The work of CR was supported by the Else Kröner-Fresenius Foundation (2016-Kolleg-19). The Neuroblastoma trial NB97 was supported by the German Cancer Aid (Grant 70-2290-Be) and by the German Children’s Cancer Aid (Grants 96.06 and 99.03). Neuroblastoma trial NB2004 was supported by the German Cancer Aid (Grant (70107712) to FB. |
---|
HeBIS-PPN: | 492046301 |
---|
Institutes: | Medizin |
---|
Dewey Decimal Classification: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
---|
Sammlungen: | Universitätspublikationen |
---|
Licence (German): | Creative Commons - Namensnennung 4.0 |
---|