title: Rhabdoid glioblastoma: an aggressive variaty of astrocytic tumor.
abstract: Rhabdoid glioblastoma (RGBM) is rare, but the most malignant among astrocytic tumors. Accumulating evidence indicates its highly aggressive nature and distinct histopathological features. Here, we report a new case of RGBM and review previously reported cases of astrocytic tumors with rhabdoid components. We describe a 58-year-old man who presented with aphasia and right-sided weakness. Magnetic resonance imaging revealed a well-delineated intramedullary tumor in the left cerebral hemisphere. Partial resection of the tumor was performed. The tumor was histologically found to contain two distinct areas: a typical glioblastoma, and a rhabdoid component. Immunohistochemical analyses revealed expression of glial fibrillary acidic protein (GFAP) and focal loss of the INI1 protein in rhabdoid cells, although fluorescence in situ hybridization analysis showed no loss of the INI1 gene. Despite subsequent radiochemotherapy for the glioblastoma, the patient died 4.3 months after surgery. Our literature review illustrates the aggressive clinical course and histopathological features of these tumors with GFAP and INI1 expression. INI1 protein dysfunction may be a possible cause of the rhabdoid phenotype. Gross total resection of the tumor and intensive radiochemotherapy may lead to better survival outcomes.
authors: Department of Neurosurgery, Sapporo Medical University School of Medicine, Sapporo, Japan ; Departments of Neurosurgery, Nagoya University Hospital, Nagoya, Japan.
authors: Momota
authors: M
authors: Hiroyuki
authors: Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan.
authors: Jiro
authors: J
authors: Ogino
authors: Departments of Neurosurgery, Sapporo Shiroishi Memorial Hospital, Sapporo, Japan.
authors: Akira
authors: A
authors: Takahashi
authors: Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan.
authors: Tadashi
authors: T
authors: Hasegawa
authors: Departments of Neurosurgery, Nagoya University Hospital, Nagoya, Japan.
authors: Toshihiko
authors: T
authors: Wakabayashi
keywords: GFAP; INI1; adult; astrocytic tumor; rhabdoid glioblastoma