CNS high-grade neuroepithelial tumor with BCOR internal tandem duplication: a comparison with its counterparts in the kidney and soft tissue

Yuka Yoshida, Sumihito Nobusawa, Satoshi Nakata, Mitsutoshi Nakada, Yoshiki Arakawa, Yohei Mineharu, Yasuo Sugita, Takako Yoshioka, Asuka Araki, Yuichiro Sato, Hideo Takeshima, Masahiko Okada, Akira Nishi, Tatsuya Yamazaki, Kenichi Kohashi, Yoshinao Oda, Junko Hirato, Hideaki Yokoo

Research output: Contribution to journalArticlepeer-review

27 Citations (Scopus)

Abstract

Central nervous system high-grade neuroepithelial tumors with BCOR alteration (CNS HGNET-BCOR) are a recently reported rare entity, identified as a small fraction of tumors previously institutionally diagnosed as so-called CNS primitive neuroectodermal tumors. Their genetic characteristic is a somatic internal tandem duplication in the 3′ end of BCOR (BCOR ITD), which has also been found in clear cell sarcomas of the kidney (CCSK) and soft tissue undifferentiated round cell sarcomas/primitive myxoid mesenchymal tumors of infancy (URCS/PMMTI), and these BCOR ITD-positive tumors have been reported to share similar pathological features. In this study, we performed a clinicopathological and molecular analysis of six cases of CNS HGNET-BCOR, and compared them with their counterparts in the kidney and soft tissue. Although these tumors had histologically similar structural patterns and characteristic monotonous nuclei with fine chromatin, CNS HGNET-BCOR exhibited glial cell morphology, ependymoma-like perivascular pseudorosettes and palisading necrosis, whereas these features were not evident in CCSK or URCS/PMMTI. Immunohistochemically, diffuse staining of Olig2 with a mixture of varying degrees of intensity, and only focal staining of GFAP, S-100 protein and synaptophysin were observed in CNS HGNET-BCOR, whereas these common neuroepithelial markers were negative in CCSK and URCS/PMMTI. Therefore, although CNS HGNET-BCOR, CCSK and URCS/PMMTI may constitute a group of BCOR ITD-positive tumors, only CNS HGNET-BCOR has histological features suggestive of glial differentiation. In conclusion, we think CNS HGNET-BCOR are a certain type of neuroepithelial tumor relatively close to glioma, not CCSK or URCS/PMMTI occurring in the CNS.

Original languageEnglish
Pages (from-to)710-720
Number of pages11
JournalBrain Pathology
Volume28
Issue number5
DOIs
Publication statusPublished - Sep 2018

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Neuroscience(all)
  • Clinical Neurology

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