The Multifaceted Appearance of Supratentorial Ependymoma with ZFTA-MAML2 Fusion

Ependymomas are glial neoplasms with a wide morphological spectrum. The majority of supratentorial ependymomas are known to harbor fusions, most commonly to . We present an unusual case of a 9-year-old boy with a supratentorial ependymoma harboring a noncanonical fusion. This case had unusual histom...

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Published in:Free Neuropathology 2021-01, Vol.2
Main Authors: Oon, Ming Liang, Hendriansyah, Lutfi, Pratiseyo, Patricia Diana, Wahjoepramono, Eka, Goh, Jian Yuan, Kuick, Chik Hong, Chang, Kenneth Te, Perry, Arie, Tan, Char Loo
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Language:English
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C11orf95
Case Report
Ependymoma
MAML2
Myogenic
ZFTA
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creator Oon, Ming Liang
Hendriansyah, Lutfi
Pratiseyo, Patricia Diana
Wahjoepramono, Eka
Goh, Jian Yuan
Kuick, Chik Hong
Chang, Kenneth Te
Perry, Arie
Tan, Char Loo
description Ependymomas are glial neoplasms with a wide morphological spectrum. The majority of supratentorial ependymomas are known to harbor fusions, most commonly to . We present an unusual case of a 9-year-old boy with a supratentorial ependymoma harboring a noncanonical fusion. This case had unusual histomorphological features lacking typical findings of ependymoma and bearing resemblance to a primitive neoplasm with focal, previously undescribed myogenic differentiation. We discuss the diagnostic pitfalls in this case and briefly review the histological features of ependymoma with noncanonical gene fusions. Our report underscores the importance of molecular testing in such cases to arrive at the correct diagnosis. Supratentorial ependymomas with noncanonical fusions are rare, and more studies are necessary for better risk stratification and identification of potential treatment targets.
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subjects C11orf95
Case Report
Ependymoma
MAML2
Myogenic
ZFTA
title The Multifaceted Appearance of Supratentorial Ependymoma with ZFTA-MAML2 Fusion
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