title: NAB2-STAT6 Gene Fusion in Meningeal Hemangiopericytoma and Solitary Fibrous Tumor.
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.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH). fritchie.karen@mayo.edu.
authors.forename: Karen J
authors.initials: KJ
authors.lastname: Fritchie
authors.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH).
authors.forename: Long
authors.initials: L
authors.lastname: Jin
authors.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH).
authors.forename: Brian P
authors.initials: BP
authors.lastname: Rubin
authors.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH).
authors.forename: Peter C
authors.initials: PC
authors.lastname: Burger
authors.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH).
authors.forename: Sarah M
authors.initials: SM
authors.lastname: Jenkins
authors.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH).
authors.forename: Sarah
authors.initials: S
authors.lastname: Barthelmeß
authors.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH).
authors.forename: Evgeny A
authors.initials: EA
authors.lastname: Moskalev
authors.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH).
authors.forename: Florian
authors.initials: F
authors.lastname: Haller
authors.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH).
authors.forename: Andre M
authors.initials: AM
authors.lastname: Oliveira
authors.affiliation: From the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota (KJF, LJ, AMO, CG); Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Molecular Genetics, Cleveland Clinic, Cleveland, Ohio (BPR); Department of Neuropathology, Johns Hopkins, Baltimore, Maryland (PCB); Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota (SMJ); and Institute of Pathology, University Hospital Erlangen, Erlangen, Germany (SB, EAM, FH).
authors.forename: Caterina
authors.initials: C
authors.lastname: Giannini
mesh_terms: D000328:Adult; D000368:Aged; D004252:DNA Mutational Analysis; D005260:Female; D050939:Gene Fusion / Q000235:genetics*; D006801:Humans; D008297:Male; D008577:Meningeal Neoplasms / Q000235:genetics* / Q000401:mortality; D008579:Meningioma / Q000235:genetics* / Q000401:mortality; D008875:Middle Aged; D012097:Repressor Proteins / Q000235:genetics* / Q000378:metabolism; D012189:Retrospective Studies; D050800:STAT6 Transcription Factor / Q000235:genetics* / Q000378:metabolism; D054364:Solitary Fibrous Tumors / Q000235:genetics* / Q000378:metabolism; D018709:Statistics, Nonparametric; D055815:Young Adult
chemical_list: C491000:NAB2 protein, human; D012097:Repressor Proteins; D050800:STAT6 Transcription Factor; C494101:STAT6 protein, human
keywords: Meningeal hemangiopericytoma; Meningeal solitary fibrous tumor; NAB2-STAT6; STAT6.