AI Article Synopsis

  • Solitary fibrous tumors (SFTs) are neoplasms originating from fusiform mesenchymal cells, primarily found in the meninges of the central nervous system, and while often benign, they can exhibit malignant behaviors in recurrences or metastases.
  • Recent research has identified the NAB2-STAT6 gene fusion as a key molecular characteristic of SFTs, which is linked to the overexpression of the NAB2-STAT6 protein in tumor cells.
  • The article highlights the importance of differentiating SFTs from hemangiopericytomas (HPC) due to distinct features that affect diagnosis, treatment, and prognosis.

Article 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.

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http://dx.doi.org/10.1016/j.neucie.2021.07.004DOI Listing

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