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Rare-43 primary central nervous system Ewing Sarcoma: a single institution experience

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dc.contributor.author Yorio, Florencia
dc.contributor.author Alessandro, Lucas
dc.contributor.author Arakaki, Naomi
dc.contributor.author Palomar, Nicolás
dc.contributor.author Muggeri, Alejandro
dc.contributor.author Diez, Blanca
dc.date.accessioned 2019-11-25T17:29:12Z
dc.date.available 2019-11-25T17:29:12Z
dc.date.issued 2019-11-11
dc.identifier.citation Yorio F, Alessandro L, Arakaki N, Palomar N, Muggeri A, Diez B. Rare-43 primary central nervous system Ewing Sarcoma: a single institution experience. Neuro Oncol. 2019;21(Supplement_6):vi230-vi231. doi:10.1093/neuonc/noz175.966 en_US
dc.identifier.uri https://doi.org/10.1093/neuonc/noz175.966
dc.identifier.uri https://repositorio.fleni.org.ar/handle/123456789/116
dc.description.abstract INTRODUCTION Ewing Sarcoma (ES) is defined by molecular markers, being t(11,22) the most frequent. Intracranial ES/pPNET usually represent metastases from extracranial sites. Primary Central Nervous System (CNS) lesions are extremely rare. MATERIAL AND METHODS Retrospective review of clinical records from patients with primary CNS ES/pPNET assessed at a neurological center in Argentina between 2007–2019. All confirmed with molecular marker. Clinical characteristics, imaging, histopathology, and treatment response were evaluated. Extensive workup included whole-body CT scan, skeletal-scintigraphy, and positron-emission tomography, excluding extracranial primary lesions in all cases. RESULTS Total 24p. Median age 22yo (2–65); M:F 2:1. Clinical presentation: intracranial hypertension, focal neurological deficit or seizures. In brain MRI 11 supratentorial lesions, 7 infratentorial and 6 diffuse leptomeningeal. Histopathology: diffuse pattern with small round blue cells 13/24p, other patterns were also described. CD99 marked positive in all cases and T(11.22) confirmed as well: 22 PCR and 2 FISH for EWSR1-rearrangement. Misdiagnosis lead to median delay for accuracy of 7,5mo (0–124); including other CNS primary tumors (n= 13), infectious diseases (n= 5) and other diagnoses (n= 4). Most patients (n= 10) were treated with Euro99-protocol (6 cycles plus local radiotherapy (RT)), 4 with Temozolomide, Irinotecan plus RT, 7 with other regimens and 2 received no treatment. Mean overall survival (OS): 58mo (0–132) and progression-free survival 22mo (0–85). Five year OS:41%. CONCLUSION Clinical behavior in primary CNS ES deferred from extraosseous ES, being necessary to apply a specific regimen for this pathology as there is no standard regime for intracranial ES/pPNET. Results showed two different groups of patients with long and very short survival. Presentation, histology, and outcomes are so uneven that we propose if a more specific molecular classification should be necessary. This is, to our knowledge, the largest case series reported describing a heterogeneous group despite the presence of accurate molecular diagnosis. en_US
dc.language.iso eng en_US
dc.publisher Oxford en_US
dc.rights info:eu-repo/semantics/openAccess
dc.rights.uri https://creativecommons.org/licenses/by/2.5/ar/
dc.subject Sarcoma, Ewing en_US
dc.subject Sarcoma de Ewing en_US
dc.subject Central Nervous System en_US
dc.subject Sistema Nervioso Central en_US
dc.title Rare-43 primary central nervous system Ewing Sarcoma: a single institution experience en_US
dc.type info:eu-repo/semantics/publishedVersion
dc.type info:eu-repo/semantics/article en_US
dc.description.fil Fil: Yorio, Florencia. Fleni. Departamento de Neurología; Argentina.
dc.description.fil Fil: Alessandro, Lucas. Fleni. Departamento de Neurología; Argentina.
dc.description.fil Fil: Arakaki, Naomi. Fleni. Departamento de Neuropatología y Biología Molecular; Argentina.
dc.description.fil Fil: Palomar, Nicolás. Fleni. Departamento de Neurología. Servicio de Neurooncología; Argentina. Hospital de Niños "Dr. Ricardo Gutiérrez"; Argentina.
dc.description.fil Fil: Muggeri, Alejandro. Fleni. Departamento de Neurología. Servicio de Neurooncología; Argentina.
dc.description.fil Fil: Diez, Blanca. Fleni. Departamento de Neurología. Servicio de Neurooncología; Argentina.
dc.relation.ispartofCOUNTRY Reino Unido
dc.relation.ispartofCITY Oxford
dc.relation.ispartofTITLE Neuro-oncology
dc.relation.ispartofISSN 1523-5866
dc.type.snrd info:ar-repo/semantics/artículo es_ES


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