神経性抗原発現を示した大脳半球神経膠芽腫の小児例

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We present an unusual pediatric case of cerebral glioblastoma expressing neuronal antigens. A seven-year-old girl was admitted to our hospital, presenting with headache and left hemiparesis. Computerized tomography scan showed an intra-axial mass with solid and cystic components in the right frontal lobe. Magnetic resonance imaging revealed that the solid component was low-intense on T1–weighted image, high-intense on T2–weighted image, and heterogeneously contrast-enhanced. The gross total removal of the tumor was performed via right high-parietal approach. Microscopically, the tumor was composed of atypical neuroepithelial cells such as small compact cells and multinucleated giant cells. Frequent mitotic figures, geographic necroses, and microvascular proliferation were also seen. The tumor cells were diffusely immunoreactive for vimentin, GFAP, Olig2, NSE and p53, but not for IDH1, NeuN, CK (CAM5.2), and p16. In addition, synaptophysin-, NFP-, and EMA- immunoreactivity were mainly recognized in some large cells. MGMT immunoreactivity was diffusely observed in small cells. MIB-1 staining index showed more than 25%. Molecular genetic analysis revealed no MGMT promoter methylation or IDH1/2, p53, H3F3A K27M, or H3F3A G34R mutations. Based on these findings, we finally diagnosed a glioblastoma with prominent expression of neuronal antigens.Although resurgery, high-dose chemotherapy with the transplantation of autologous peripheral blood stem cell, and craniospinal irradiation were performed, the tumor was early recurred. After all, she died 14 months after the first surgery.Further detailed investigation should be required for the understanding of the expression of the neuronal antigens in glioblastomas.
近畿脳腫瘍病理検討会の論文

神経性抗原発現を示した大脳半球神経膠芽腫の小児例

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