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

• Published in: Brain tumor pathology 2015-10, Vol.32 (4), p.268-274
• Main Authors: Nakada, Satoko, Minato, Hiroshi, Takegami, Tsutomu, Kurose, Nozomu, Ikeda, Hiroko, Kobayashi, Masako, Sasagawa, Yasuo, Akai, Takuya, Kato, Takashi, Yamamoto, Norio, Nojima, Takayuki
• Format: Article
• Language: English
• Subjects: Adult; Aged; Autopsies; Bone Neoplasms - genetics; Bone Neoplasms - secondary; Cancer Research; Case Report; Cerebellar Neoplasms - genetics; Cerebellar Neoplasms - pathology; Cloning; Cranial Fossa, Middle; Exons; Female; Gene Fusion; Genes; Hemangiopericytoma - genetics; Hemangiopericytoma - secondary; Histology; Humans; Immunohistochemistry; Keratin; Lung Neoplasms - genetics; Lung Neoplasms - secondary; Male; Medicine; Medicine & Public Health; Metastasis; Neurology; Neurosurgery; Oncology; Pathology; Radiation; Repressor Proteins - analysis; Repressor Proteins - genetics; Skull Base Neoplasms - genetics; Skull Base Neoplasms - pathology; STAT6 Transcription Factor - analysis; STAT6 Transcription Factor - genetics; Tumors
• ISSN: 1433-7398; 1861-387X
• DOI: 10.1007/s10014-015-0220-x

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.