NAB2-STAT6 Gene Fusion in Meningeal Hemangiopericytoma and Solitary Fibrous Tumor

Fritchie KJ, Jin L, Rubin BP, Burger PC, Jenkins SM, Barthelmeß S, Moskalev E, Haller F, Oliveira AM, Giannini C (2016)

Publication Type: Journal article

Publication year: 2016

Journal

Journal of Neuropathology and Experimental Neurology Oxford University Press

Book Volume: 75

Pages Range: 263-71

Journal Issue: 3

DOI: 10.1093/jnen/nlv026

Abstract

Meningeal solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) are considered to be distinct entities in the WHO Classification of CNS Tumours (2007). They harbor NAB2-STAT6 fusions similar to their soft tissue counterparts, supporting the view that they are part of a tumor continuum. We examined 30 meningeal-based tumors originally diagnosed as either SFT or HPC. These showed a spectrum of morphologic features and were diagnosed as SFTs, malignant SFTs, HPCs, or tumors with "intermediate" features. All of the tumors showed nuclear expression of STAT6. SFTs consistently expressed diffuse CD34, while HPCs and intermediate tumors had heterogeneous staining. NAB2-STAT6 fusions were identified in 20 cases, including 7 with exon 4-exon 3, 9 with exon 6-exon 17, and 4 with exon 6-exon 18 fusions. NAB2 exon 4-STAT6 exon 3 fusion correlated with classic SFT morphology and older age and showed a trend toward less mitotic activity; there was also a trend toward more aggressive behavior in tumors lacking NAB2 exon 4-STAT6 exon 3. Thus, despite their clinical and morphologic differences, meningeal-based SFTs, HPCs, and tumors with intermediate features, similar to their soft tissue counterparts, form a histopathologic spectrum unified by STAT6 immunoexpression and NAB2-STAT6 fusion.

Authors with CRIS profile

Sarah Barthelmeß Institute of Pathology Evgeny Moskalev Institute of Pathology

Involved external institutions

Mayo Clinic US United States (USA) (US) Johns Hopkins University (JHU) US United States (USA) (US) Cleveland Clinic US United States (USA) (US)

How to cite

APA:

Fritchie, K.J., Jin, L., Rubin, B.P., Burger, P.C., Jenkins, S.M., Barthelmeß, S.,... Giannini, C. (2016). NAB2-STAT6 Gene Fusion in Meningeal Hemangiopericytoma and Solitary Fibrous Tumor. Journal of Neuropathology and Experimental Neurology, 75(3), 263-71. https://doi.org/10.1093/jnen/nlv026

MLA:

Fritchie, Karen J., et al. "NAB2-STAT6 Gene Fusion in Meningeal Hemangiopericytoma and Solitary Fibrous Tumor." Journal of Neuropathology and Experimental Neurology 75.3 (2016): 263-71.

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