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Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity

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Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. / Korshunov, Andrey; Sturm, Dominik; Ryzhova, Marina; Hovestadt, Volker; Gessi, Marco; Jones, David T W; Remke, Marc; Northcott, Paul A; Perry, Arie; Picard, Daniel; Rosenblum, Marc; Antonelli, Manila; Aronica, Eleonora; Schüller, Ulrich; Hasselblatt, Martin; Woehrer, Adelheid; Zheludkova, Olga; Kumirova, Ella; Puget, Stephanie; Taylor, Michael D; Giangaspero, Felice; Peter Collins, V; Deimling, Andreas; Lichter, Peter; Huang, Annie; Pietsch, Torsten; Pfister, Stefan M; Kool, Marcel.

In: ACTA NEUROPATHOL, Vol. 128, No. 2, 08.2014, p. 279-89.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Korshunov, A, Sturm, D, Ryzhova, M, Hovestadt, V, Gessi, M, Jones, DTW, Remke, M, Northcott, PA, Perry, A, Picard, D, Rosenblum, M, Antonelli, M, Aronica, E, Schüller, U, Hasselblatt, M, Woehrer, A, Zheludkova, O, Kumirova, E, Puget, S, Taylor, MD, Giangaspero, F, Peter Collins, V, Deimling, A, Lichter, P, Huang, A, Pietsch, T, Pfister, SM & Kool, M 2014, 'Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity', ACTA NEUROPATHOL, vol. 128, no. 2, pp. 279-89. https://doi.org/10.1007/s00401-013-1228-0

APA

Korshunov, A., Sturm, D., Ryzhova, M., Hovestadt, V., Gessi, M., Jones, D. T. W., Remke, M., Northcott, P. A., Perry, A., Picard, D., Rosenblum, M., Antonelli, M., Aronica, E., Schüller, U., Hasselblatt, M., Woehrer, A., Zheludkova, O., Kumirova, E., Puget, S., ... Kool, M. (2014). Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. ACTA NEUROPATHOL, 128(2), 279-89. https://doi.org/10.1007/s00401-013-1228-0

Vancouver

Korshunov A, Sturm D, Ryzhova M, Hovestadt V, Gessi M, Jones DTW et al. Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. ACTA NEUROPATHOL. 2014 Aug;128(2):279-89. https://doi.org/10.1007/s00401-013-1228-0

Bibtex

@article{32eef8c7aff441f58ef1fa392e16b72e,
title = "Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity",
abstract = "Three histological variants are known within the family of embryonal rosette-forming neuroepithelial brain tumors. These include embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL), and medulloepithelioma (MEPL). In this study, we performed a comprehensive clinical, pathological, and molecular analysis of 97 cases of these rare brain neoplasms, including genome-wide DNA methylation and copy number profiling of 41 tumors. We identified uniform molecular signatures in all tumors irrespective of histological patterns, indicating that ETANTR, EBL, and MEPL comprise a single biological entity. As such, future WHO classification schemes should consider lumping these variants into a single diagnostic category, such as embryonal tumor with multilayered rosettes (ETMR). We recommend combined LIN28A immunohistochemistry and FISH analysis of the 19q13.42 locus for molecular diagnosis of this tumor category. Recognition of this distinct pediatric brain tumor entity based on the fact that the three histological variants are molecularly and clinically uniform will help to distinguish ETMR from other embryonal CNS tumors and to better understand the biology of these highly aggressive and therapy-resistant pediatric CNS malignancies, possibly leading to alternate treatment strategies.",
keywords = "Brain Neoplasms, Child, Child, Preschool, Chromosomes, Human, Pair 19, DNA Copy Number Variations, DNA Methylation, Diagnosis, Differential, Female, Genetic Loci, Humans, Immunohistochemistry, In Situ Hybridization, Fluorescence, Infant, Male, Neoplasm Recurrence, Local, Neoplasms, Germ Cell and Embryonal, Neuroectodermal Tumors, Primitive, Survival Analysis, Journal Article",
author = "Andrey Korshunov and Dominik Sturm and Marina Ryzhova and Volker Hovestadt and Marco Gessi and Jones, {David T W} and Marc Remke and Northcott, {Paul A} and Arie Perry and Daniel Picard and Marc Rosenblum and Manila Antonelli and Eleonora Aronica and Ulrich Sch{\"u}ller and Martin Hasselblatt and Adelheid Woehrer and Olga Zheludkova and Ella Kumirova and Stephanie Puget and Taylor, {Michael D} and Felice Giangaspero and {Peter Collins}, V and Andreas Deimling and Peter Lichter and Annie Huang and Torsten Pietsch and Pfister, {Stefan M} and Marcel Kool",
year = "2014",
month = aug,
doi = "10.1007/s00401-013-1228-0",
language = "English",
volume = "128",
pages = "279--89",
journal = "ACTA NEUROPATHOL",
issn = "0001-6322",
publisher = "Springer",
number = "2",

}

RIS

TY - JOUR

T1 - Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity

AU - Korshunov, Andrey

AU - Sturm, Dominik

AU - Ryzhova, Marina

AU - Hovestadt, Volker

AU - Gessi, Marco

AU - Jones, David T W

AU - Remke, Marc

AU - Northcott, Paul A

AU - Perry, Arie

AU - Picard, Daniel

AU - Rosenblum, Marc

AU - Antonelli, Manila

AU - Aronica, Eleonora

AU - Schüller, Ulrich

AU - Hasselblatt, Martin

AU - Woehrer, Adelheid

AU - Zheludkova, Olga

AU - Kumirova, Ella

AU - Puget, Stephanie

AU - Taylor, Michael D

AU - Giangaspero, Felice

AU - Peter Collins, V

AU - Deimling, Andreas

AU - Lichter, Peter

AU - Huang, Annie

AU - Pietsch, Torsten

AU - Pfister, Stefan M

AU - Kool, Marcel

PY - 2014/8

Y1 - 2014/8

N2 - Three histological variants are known within the family of embryonal rosette-forming neuroepithelial brain tumors. These include embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL), and medulloepithelioma (MEPL). In this study, we performed a comprehensive clinical, pathological, and molecular analysis of 97 cases of these rare brain neoplasms, including genome-wide DNA methylation and copy number profiling of 41 tumors. We identified uniform molecular signatures in all tumors irrespective of histological patterns, indicating that ETANTR, EBL, and MEPL comprise a single biological entity. As such, future WHO classification schemes should consider lumping these variants into a single diagnostic category, such as embryonal tumor with multilayered rosettes (ETMR). We recommend combined LIN28A immunohistochemistry and FISH analysis of the 19q13.42 locus for molecular diagnosis of this tumor category. Recognition of this distinct pediatric brain tumor entity based on the fact that the three histological variants are molecularly and clinically uniform will help to distinguish ETMR from other embryonal CNS tumors and to better understand the biology of these highly aggressive and therapy-resistant pediatric CNS malignancies, possibly leading to alternate treatment strategies.

AB - Three histological variants are known within the family of embryonal rosette-forming neuroepithelial brain tumors. These include embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL), and medulloepithelioma (MEPL). In this study, we performed a comprehensive clinical, pathological, and molecular analysis of 97 cases of these rare brain neoplasms, including genome-wide DNA methylation and copy number profiling of 41 tumors. We identified uniform molecular signatures in all tumors irrespective of histological patterns, indicating that ETANTR, EBL, and MEPL comprise a single biological entity. As such, future WHO classification schemes should consider lumping these variants into a single diagnostic category, such as embryonal tumor with multilayered rosettes (ETMR). We recommend combined LIN28A immunohistochemistry and FISH analysis of the 19q13.42 locus for molecular diagnosis of this tumor category. Recognition of this distinct pediatric brain tumor entity based on the fact that the three histological variants are molecularly and clinically uniform will help to distinguish ETMR from other embryonal CNS tumors and to better understand the biology of these highly aggressive and therapy-resistant pediatric CNS malignancies, possibly leading to alternate treatment strategies.

KW - Brain Neoplasms

KW - Child

KW - Child, Preschool

KW - Chromosomes, Human, Pair 19

KW - DNA Copy Number Variations

KW - DNA Methylation

KW - Diagnosis, Differential

KW - Female

KW - Genetic Loci

KW - Humans

KW - Immunohistochemistry

KW - In Situ Hybridization, Fluorescence

KW - Infant

KW - Male

KW - Neoplasm Recurrence, Local

KW - Neoplasms, Germ Cell and Embryonal

KW - Neuroectodermal Tumors, Primitive

KW - Survival Analysis

KW - Journal Article

U2 - 10.1007/s00401-013-1228-0

DO - 10.1007/s00401-013-1228-0

M3 - SCORING: Journal article

C2 - 24337497

VL - 128

SP - 279

EP - 289

JO - ACTA NEUROPATHOL

JF - ACTA NEUROPATHOL

SN - 0001-6322

IS - 2

ER -