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Vol. 33. Issue 6.
Pages 371-376 (November - December 2022)
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Vol. 33. Issue 6.
Pages 371-376 (November - December 2022)
Case Report
Recurrent meningeal malignant tumor: Assessment of differences in the solitary fibrous tumor/hemangiopericytoma spectrum through a case report
Tumor maligno recurrente de origen meníngeo: evaluación de las diferencias de la entidad tumor fibroso solitario/hemangiopericitoma mediante un caso clínico
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Carlos Sánchez Fernándeza,
Corresponding author
carlos_san_fer@hotmail.com

Corresponding author.
, Elena García Lagartob, Carlos Alberto Rodríguez-Ariasa
a Department of Neurosurgery, University Clinical Hospital of Valladolid, Valladolid, Spain
b Department of Pathology, University Clinical Hospital of Valladolid, Valladolid, Spain
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Abstract

Solitary fibrous tumors (SFTs) are neoplasms that grow from mesenchymal fusiform cells. In the central nervous system, meninges are the common origin of these neoplasms. Although literature reports mostly SFT as benign neoplasm, malignancy data have been described in recurrences or metastatic lesions. Definitive diagnosis includes immunohistochemical profiles assessing cellular positivity for CD34, vimentin, CD99 and Bcl-2. Recent studies have demonstrated NAB2-STAT6 gene fusion as a distinct molecular feature of SFT with overexpression of the fusion protein NAB2-STAT6 in nuclei of these cells. Since several years, pathologists have grouped SFT and hemangiopericytomas (HPC) as different phenotypes of the same entity although both neoplasms do not share numerous features. This article, based on a case of a recurrent malignant SFT, aims to emphasize differences in the SFT/HPC spectrum due to the diagnostic, therapeutic and prognostic implications.

Keywords:
Hemangiopericytoma
Intracranial tumour
Mesenchymal neoplasm
Solitary fibrous tumour
Surgical excision
Resumen

Los tumores fibrosos solitarios (TFS) son neoplasias que crecen a partir de células mesenquimales y las meninges constituyen su origen preferente en el sistema nervioso central. Aunque la literatura relaciona la mayoría de los TFS como neoplasias benignas, se describen datos de malignidad en recidivas tumorales o lesiones metastásicas. El diagnóstico definitivo incluye el perfil inmunohistoquímico, que evalúa la positividad celular para CD34, vimentina, CD99 y Bcl-2. Estudios recientes han demostrado la fusión del gen NAB2-STAT6 como una característica molecular distintiva de los TFS, con sobreexpresión de la proteína de fusión NAB2-STAT6 en los núcleos de las células. Los patólogos han agrupado los TFS y los hemangiopericitomas como diferentes fenotipos de una misma entidad, aunque ambas neoplasias no comparten numerosas características. Este artículo, basado en un caso de una lesión maligna recurrente, tiene como objetivo enfatizar las diferencias en el espectro SFT/hemangiopericitoma por sus implicaciones diagnósticas, terapéuticas y pronósticas.

Palabras clave:
Hemangiopericitoma
Tumor intracraneal
Neoplasia mesenquimal
Tumor fibroso solitario
Resección quirúrgica

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