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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-64879

von Bueren, André O; Gerss, Joachim; Hagel, Christian; Cai, Haoyang; Remke, Marc; Hasselblatt, Martin; Feuerstein, Burt G; Pernet, Sarah; Delattre, Olivier; Korshunov, Andrey; Rutkowski, Stefan; Pfister, Stefan M; Baudis, Michael (2012). DNA copy number alterations in central primitive neuroectodermal tumors and tumors of the pineal region: an international individual patient data meta-analysis. Journal of Neuro-Oncology, 109(2):415-423.

Accepted Version


Little is known about frequency, association with clinical characteristics, and prognostic impact of DNA copy number alterations (CNA) on survival in central primitive neuroectodermal tumors (CNS-PNET) and tumors of the pineal region. Searches of MEDLINE, Pubmed, and EMBASE-after the original description of comparative genomic hybridization in 1992 and July 2010-identified 15 case series of patients with CNS-PNET and tumors of the pineal region whose tumors were investigated for genome-wide CNA. One additional case study was identified from contact with experts. Individual patient data were extracted from publications or obtained from investigators, and CNAs were converted to a digitized format suitable for data mining and subgroup identification. Summary profiles for genomic imbalances were generated from case-specific data. Overall survival (OS) was estimated using the Kaplan-Meier method, and by univariable and multivariable Cox regression models. In their overall CNA profiles, low grade tumors of the pineal region clearly diverged from CNS-PNET and pineoblastoma. At a median follow-up of 89 months, 7-year OS rates of CNS-PNET, pineoblastoma, and low grade tumors of the pineal region were 22.9 ± 6, 0 ± 0, and 87.5 ± 12 %, respectively. Multivariable analysis revealed that histology (CNS-PNET), age (≤2.5 years), and possibly recurrent CNAs were associated with unfavorable OS. DNA copy number profiling suggests a close relationship between CNS-PNET and pineoblastoma. Low grade tumors of the pineal region differed from CNS-PNET and pineoblastoma. Due to their high biological and clinical variability, a coordinated prospective validation in future studies is necessary to establish robust risk factors.

Item Type:Journal Article, refereed, original work
Communities & Collections:07 Faculty of Science > Institute of Molecular Life Sciences
DDC:570 Life sciences; biology
Date:7 July 2012
Deposited On:26 Sep 2012 15:38
Last Modified:27 Nov 2013 23:46
Additional Information:The original publication is available at www.springerlink.com
Publisher DOI:10.1007/s11060-012-0911-7
PubMed ID:22772606
Citations:Web of Science®. Times Cited: 2
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Scopus®. Citation Count: 5

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