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MYCN amplification predicts poor outcome for patients with supratentorial primitive neuroectodermal tumors of the central nervous system

Standard

MYCN amplification predicts poor outcome for patients with supratentorial primitive neuroectodermal tumors of the central nervous system. / Gessi, Marco; von Bueren, André O; Treszl, Andras; Mühlen, Anja Zur; Hartmann, Wolfgang; Warmuth-Metz, Monika; Rutkowski, Stefan; Pietsch, Torsten.

in: NEURO-ONCOLOGY, Jahrgang 16, Nr. 7, 26.01.2014, S. 924-932.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Gessi, M, von Bueren, AO, Treszl, A, Mühlen, AZ, Hartmann, W, Warmuth-Metz, M, Rutkowski, S & Pietsch, T 2014, 'MYCN amplification predicts poor outcome for patients with supratentorial primitive neuroectodermal tumors of the central nervous system', NEURO-ONCOLOGY, Jg. 16, Nr. 7, S. 924-932. https://doi.org/10.1093/neuonc/not302

APA

Gessi, M., von Bueren, A. O., Treszl, A., Mühlen, A. Z., Hartmann, W., Warmuth-Metz, M., Rutkowski, S., & Pietsch, T. (2014). MYCN amplification predicts poor outcome for patients with supratentorial primitive neuroectodermal tumors of the central nervous system. NEURO-ONCOLOGY, 16(7), 924-932. https://doi.org/10.1093/neuonc/not302

Vancouver

Gessi M, von Bueren AO, Treszl A, Mühlen AZ, Hartmann W, Warmuth-Metz M et al. MYCN amplification predicts poor outcome for patients with supratentorial primitive neuroectodermal tumors of the central nervous system. NEURO-ONCOLOGY. 2014 Jan 26;16(7):924-932. https://doi.org/10.1093/neuonc/not302

Bibtex

@article{f3aa4519b831467a84b0ff2ef204d035,
title = "MYCN amplification predicts poor outcome for patients with supratentorial primitive neuroectodermal tumors of the central nervous system",
abstract = "BackgroundPrimitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are a rare group of neoplasms occurring in the CNS that includes supratentorial CNS-PNETs, medulloepitheliomas, and ependymoblastomas. While ependymoblastomas frequently carry chromosome 19q13.41 amplification and show aggressive clinical behavior, the biological mechanisms and molecular alterations contributing to the pathogenesis of supratentorial CNS-PNETs remain poorly understood. Moreover, genetic alterations suitable for molecular risk stratification are undefined to date.MethodsIn order to identify possible molecular markers, we performed multiplex ligation-dependent probe amplification (MLPA) and molecular inversion probe (MIP) analysis on DNA samples of 25 supratentorial CNS-PNETs (median age, 5.35 years; range, 2.41-17.28 years). Tumors with ependymoblastic rosettes (ependymoblastoma/ETANTR) and LIN28A positivity were excluded.ResultsMLPA and MIP analysis revealed large losses of genomic material of chromosomes 3, 4, 5, and 13, while frequent gains affected chromosomes 1, 17, 19, 20, and 22. High copy number gains (amplifications) were found in particular at chromosomes 2p24.3 (MYCN, n = 6 cases) and 4q12 (n = 2 cases). Patients with tumors harboring 2p gain or MYCN amplification showed unfavorable overall survival (P = .003 and P = .001, respectively).These markers were independent of the presence of metastases, which was indeed a clinical factor associated with poor overall survival (P = .01) in this series.ConclusionsIn the era of the personalized neuro-oncology, the identification of these molecular prognostic markers associated with patient outcome may represent a significant step towards improved patient stratification and risk-adapted therapeutic strategies for patients suffering from supratentorial CNS-PNETs.",
author = "Marco Gessi and {von Bueren}, {Andr{\'e} O} and Andras Treszl and M{\"u}hlen, {Anja Zur} and Wolfgang Hartmann and Monika Warmuth-Metz and Stefan Rutkowski and Torsten Pietsch",
year = "2014",
month = jan,
day = "26",
doi = "10.1093/neuonc/not302",
language = "English",
volume = "16",
pages = "924--932",
journal = "NEURO-ONCOLOGY",
issn = "1522-8517",
publisher = "Oxford University Press",
number = "7",

}

RIS

TY - JOUR

T1 - MYCN amplification predicts poor outcome for patients with supratentorial primitive neuroectodermal tumors of the central nervous system

AU - Gessi, Marco

AU - von Bueren, André O

AU - Treszl, Andras

AU - Mühlen, Anja Zur

AU - Hartmann, Wolfgang

AU - Warmuth-Metz, Monika

AU - Rutkowski, Stefan

AU - Pietsch, Torsten

PY - 2014/1/26

Y1 - 2014/1/26

N2 - BackgroundPrimitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are a rare group of neoplasms occurring in the CNS that includes supratentorial CNS-PNETs, medulloepitheliomas, and ependymoblastomas. While ependymoblastomas frequently carry chromosome 19q13.41 amplification and show aggressive clinical behavior, the biological mechanisms and molecular alterations contributing to the pathogenesis of supratentorial CNS-PNETs remain poorly understood. Moreover, genetic alterations suitable for molecular risk stratification are undefined to date.MethodsIn order to identify possible molecular markers, we performed multiplex ligation-dependent probe amplification (MLPA) and molecular inversion probe (MIP) analysis on DNA samples of 25 supratentorial CNS-PNETs (median age, 5.35 years; range, 2.41-17.28 years). Tumors with ependymoblastic rosettes (ependymoblastoma/ETANTR) and LIN28A positivity were excluded.ResultsMLPA and MIP analysis revealed large losses of genomic material of chromosomes 3, 4, 5, and 13, while frequent gains affected chromosomes 1, 17, 19, 20, and 22. High copy number gains (amplifications) were found in particular at chromosomes 2p24.3 (MYCN, n = 6 cases) and 4q12 (n = 2 cases). Patients with tumors harboring 2p gain or MYCN amplification showed unfavorable overall survival (P = .003 and P = .001, respectively).These markers were independent of the presence of metastases, which was indeed a clinical factor associated with poor overall survival (P = .01) in this series.ConclusionsIn the era of the personalized neuro-oncology, the identification of these molecular prognostic markers associated with patient outcome may represent a significant step towards improved patient stratification and risk-adapted therapeutic strategies for patients suffering from supratentorial CNS-PNETs.

AB - BackgroundPrimitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are a rare group of neoplasms occurring in the CNS that includes supratentorial CNS-PNETs, medulloepitheliomas, and ependymoblastomas. While ependymoblastomas frequently carry chromosome 19q13.41 amplification and show aggressive clinical behavior, the biological mechanisms and molecular alterations contributing to the pathogenesis of supratentorial CNS-PNETs remain poorly understood. Moreover, genetic alterations suitable for molecular risk stratification are undefined to date.MethodsIn order to identify possible molecular markers, we performed multiplex ligation-dependent probe amplification (MLPA) and molecular inversion probe (MIP) analysis on DNA samples of 25 supratentorial CNS-PNETs (median age, 5.35 years; range, 2.41-17.28 years). Tumors with ependymoblastic rosettes (ependymoblastoma/ETANTR) and LIN28A positivity were excluded.ResultsMLPA and MIP analysis revealed large losses of genomic material of chromosomes 3, 4, 5, and 13, while frequent gains affected chromosomes 1, 17, 19, 20, and 22. High copy number gains (amplifications) were found in particular at chromosomes 2p24.3 (MYCN, n = 6 cases) and 4q12 (n = 2 cases). Patients with tumors harboring 2p gain or MYCN amplification showed unfavorable overall survival (P = .003 and P = .001, respectively).These markers were independent of the presence of metastases, which was indeed a clinical factor associated with poor overall survival (P = .01) in this series.ConclusionsIn the era of the personalized neuro-oncology, the identification of these molecular prognostic markers associated with patient outcome may represent a significant step towards improved patient stratification and risk-adapted therapeutic strategies for patients suffering from supratentorial CNS-PNETs.

U2 - 10.1093/neuonc/not302

DO - 10.1093/neuonc/not302

M3 - SCORING: Journal article

C2 - 24470553

VL - 16

SP - 924

EP - 932

JO - NEURO-ONCOLOGY

JF - NEURO-ONCOLOGY

SN - 1522-8517

IS - 7

ER -