Primary intracranial Ewing sarcoma/ peripheral primitive neuroectodermal tumor, an entity of unacquaintance: a series of
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ORIGINAL ARTICLE
Primary intracranial Ewing sarcoma/ peripheral primitive neuroectodermal tumor, an entity of unacquaintance: a series of 8 cases Gauri Deshpande 1 & Sridhar Epari 1 Tejpal Gupta 3
&
Chhavi Gupta 1 & Omshree Shetty 1 & Mamta Gurav 1 & Girish Chinnaswamy 2 &
Received: 30 March 2020 / Accepted: 30 July 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
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. Keywords Ewing sarcoma . Peripheral primitive neuroectodermal tumor . Meninges . EWSR1 gene rearrangement . CD99
Introduction
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00381-020-04850-w) contains supplementary material, which is available to authorized users. * Sridhar Epari [email protected] 1
Department of Pathology (& Division of Molecular Pathology), Tata Memorial Hospital and ACTREC, Tata Memorial Centre, Homi Bhabha National Institute, 8th Floor, Annexe Building, Dr. E. Borges Road, Parel, Mumbai 400012, India
2
Department of Pediatric Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India
3
Department of Radiation Oncology, Tata Memorial Hospital and ACTREC, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India
Ewing sarcoma/primitive neuroectodermal tumors (ES/ PNETs) are a distinct family of embryonal tumors with a conspicuous immunophenotypic finding of diffuse cytoplasmic membrane staining for CD99 and characteristic cytogenetic abnormality of EWSR1 gene rearrangement of specific translocation with any of the members of ETS family of transcription fac
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