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Published in: Child's Nervous System 3/2021

01-03-2021 | Computed Tomography | Original Article

Primary intracranial Ewing sarcoma/ peripheral primitive neuroectodermal tumor, an entity of unacquaintance: a series of 8 cases

Authors: Gauri Deshpande, Sridhar Epari, Chhavi Gupta, Omshree Shetty, Mamta Gurav, Girish Chinnaswamy, Aliasagar Moiyadi, Tejpal Gupta

Published in: Child's Nervous System | Issue 3/2021

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Abstract

Purpose

The purpose is to highlight the primary intracranial (meningeal-based) occurrence of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET).

Methods

This report is a collation of clinicopathological features of eight cases of molecularly and clinicoradiologically confirmed primary (non-metastatic) intracranial (non-osseous) meningeal ES/PNET.

Results

The age range was 1 to 33 years with a median age of 9 years. Male to female ratio was 0.6:1. All patients were diagnosed on the debulking surgical material (gross total resection, 2 cases; subtotal resection, 6 cases) and showed primitive embryonal histomorphology with diffuse membranous CD99 immunoexpression and EWSR1 gene rearrangement by fluorescence in situ hybridization. Seven of them showed a typical FISH pattern of split signals with break-apart probe, while one showed an unusual signal pattern of loss of green signals. EFT-2001 adjuvant protocol was followed along with focal radiotherapy (RT) in all cases (except case 8, full course of chemotherapy could not be completed). Two cases had local recurrence—one of them died of disease recurrence before the administration of further treatment.

Conclusion

This series adds non-osseous intracranial site to the list of uncommon sites of occurrence for ES/PNET and more importantly emphasizes the need to be considered in a differential list of primary intracranial primitive embryonal tumors before embarking as primary central nervous system (CNS) embryonal tumor, NOS.
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Metadata
Title
Primary intracranial Ewing sarcoma/ peripheral primitive neuroectodermal tumor, an entity of unacquaintance: a series of 8 cases
Authors
Gauri Deshpande
Sridhar Epari
Chhavi Gupta
Omshree Shetty
Mamta Gurav
Girish Chinnaswamy
Aliasagar Moiyadi
Tejpal Gupta
Publication date
01-03-2021
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 3/2021
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-020-04850-w

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