NAB2-STAT6 fusion gene analysis in two cases of meningeal solitary fibrous tumor/hemangiopericytoma with late distant metastases

Brain Tumor Pathol. 2015 Oct;32(4):268-74. doi: 10.1007/s10014-015-0220-x. Epub 2015 Apr 18.

Abstract

We present two cases of meningeal solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) with immunohistochemistry of STAT6 and analysis of NAB2-STAT6 fusion genes. Case 1 was a 37-year-old male with a left middle fossa tumor; case 2 was a 68-year-old female with a cerebellar tumor. They showed late metastasis to the lung or bone 8 or 13 years, respectively, after the first surgery. Histology of both primary and metastatic tumors showed a cellular hemangiopericytomatous pattern with nuclear atypia. The primary tumors showed nuclear staining of STAT6, but both metastatic tumors showed nuclear and cytoplasmic STAT6. DNA sequencing revealed two kinds of NAB2-STAT6 fusion genes. One consisted of exon 6 of NAB2, intron 6 of NAB2, and the middle of exon 17 of STAT6 (observed in the primary and metastatic tumors of case 1); the other consisted of exon 6 of NAB2 and the beginning of exon 17 of STAT6 (observed in the metastatic tumor of case 2). The primary tumor of case 2 had both fusion genes. To the best of our knowledge, we are the first to report NAB2-STAT6 fusion gene analysis in primary and metastatic meningeal SFT/HPCs and a case showed different fusion gene status in the metastatic tumor.

Keywords: Hemangiopericytoma; Immunohistochemistry; Meningeal tumor; NAB2–STAT6 fusion gene; Solitary fibrous tumor.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Bone Neoplasms / genetics
  • Bone Neoplasms / secondary
  • Cerebellar Neoplasms / genetics*
  • Cerebellar Neoplasms / pathology*
  • Cranial Fossa, Middle
  • Exons
  • Female
  • Gene Fusion*
  • Hemangiopericytoma / genetics*
  • Hemangiopericytoma / secondary*
  • Humans
  • Immunohistochemistry
  • Lung Neoplasms / genetics
  • Lung Neoplasms / secondary
  • Male
  • Repressor Proteins / analysis
  • Repressor Proteins / genetics*
  • STAT6 Transcription Factor / analysis
  • STAT6 Transcription Factor / genetics*
  • Skull Base Neoplasms / genetics*
  • Skull Base Neoplasms / pathology*

Substances

  • NAB2 protein, human
  • Repressor Proteins
  • STAT6 Transcription Factor
  • STAT6 protein, human