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Extraventricular neurocytoma (EVN) is a rare intracranial tumor. The most common locations of EVN were frontal lobe and cerebellum, followed by temporal lobe. Heterogeneous contrast enhancement, cystic changes, perilesional edema and calcification were a few distinct imaging characteristics. EVN is a primary tumor with either glial or neuronal differentiation or potential for atypical changes. The lack of characteristic manifestations in histopathology makes EVN difficult to distinguish from other low-grade neuronal and mixed neuronal-glial tumors. Herein, we describe a case of frontal extraventricular neurocytoma in a 37-year-old male presenting with dizzy and review the literature on this rare tumor. Pathological examination reveals that the tumor mainly composed of oligodendroglia-like cells, with expression of synaptophysin (SYN) and Olig2 (oligodendrocyte transcription factor 2), and microcalcifications. Methylation profile and t-SNE analysis show our sample cluster with the other two reference EVNs. Next-generation sequencing (NGS) shows FGFR1-TACC1 fusion. We report a case of EVN with expression of Olig2, FGFR1-TACC1 fusion, methylation of MGMT promoter, and discuss our experience along with a review of the published literature.

Background: Brain metastases (BMs) are the most common central nervous system (CNS) malignant tumors, with rapid disease progression and extremely poor prognosis. The heterogeneity between primary tumors and BMs leads to the divergent efficacy of the adjuvant therapy response to primary tumors and BMs. However, the extent of heterogeneity between primary tumors and BMs, and the evolutionary process remains little known. Methods: To deeply insight the extent of inter-tumor heterogeneity at single-patient level and the process of these evolutions, we retrospectively analyzed a total of 26 tumor samples from 11 patients with matched primary tumors and BMs. One patient underwent four times brain metastatic lesion surgery with diverse locations and one operation for the primary lesion. The genomic and immune heterogeneity between primary tumors and BMs was evaluated by utilizing the whole-exome sequencing (WESeq) and immunohistochemical analysis.Results: In addition to inheriting genomic phenotype and molecular phenotype from the primary tumors, massive unique genomic phenotype and molecular phenotype were also observed in BMs, which revealed unimaginable complexity of tumor evolution and extensive heterogeneity among lesions at single-patient level. Our study also verified that the expression level of immune checkpoints-related molecule Programmed Death-Ligand 1 (PD-L1) (P = 0.0013) and the density of tumor-infiltrating lymphocytes (TILs) (P = 0.0248) in BMs were significantly lower than that in paired primary tumors. Additionally, tumor microvascular density (MVD) and tumor invasiveness were also differed between primary tumors and paired BMs, indicating that temporal and spatial diversity profoundly contributes to the evolution of BMs heterogeneity.Conclusion: We verified the significance of temporal and spatial factors to the evolution of tumor heterogeneity by multi-dimensional analysis of matched primary tumors and BMs, which also provided novel insight for formulating individualized treatment strategies of BMs.