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

• Published in: Child's nervous system 2021-03, Vol.37 (3), p.839-849
• Main Authors: Deshpande, Gauri, Epari, Sridhar, Gupta, Chhavi, Shetty, Omshree, Gurav, Mamta, Chinnaswamy, Girish, Moiyadi, Aliasagar, Gupta, Tejpal
• Format: Article
• Language: English
• Subjects: Medicine; Medicine & Public Health; Neurosciences; Neurosurgery; Original Article
• ISSN: 0256-7040; 1433-0350
• DOI: 10.1007/s00381-020-04850-w

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.