Section of Neurosurgery, Department of Clinical Neuroscience, Faculty of Medicine, University of Miyazaki, 5200 Kihara Kiyotake, Miyazaki 889-1692, Japan.
Brain Tumor Pathol. 2011 Oct;28(4):311-6. doi: 10.1007/s10014-011-0047-z. Epub 2011 Jul 2.
Gangliogliomas are rare tumors of the central nervous system, usually containing neoplastic ganglion cells and astrocytic components. Few cases of ganglioglioma containing only oligodendrocytic tissue have been reported to date. We present a case of a 40-year-old woman with ganglioglioma consisting mostly of oligodendroglial components. Magnetic resonance imaging showed a well-demarcated cystic lesion with slight perifocal edema in the right parietal lobe. The wall of the cyst was not enhanced after administration of Gd-DTPA contrast media. The mass was totally resected. Histological examination showed a mixture of two distinct components: oligodendroglioma and dysplastic ganglions. The first component was diffusely proliferated cells with round nuclei and perinuclear halo; the second showed marked nucleoli and basophilic cytoplasm containing Nissl bodies. Immunohistochemical study of the oligodendroglial component was positive for OLIG 2 and NKX2.2 but negative for synaptophysin. In addition, LOH of 1p/19q was detected by FISH. Although no adjuvant therapy was carried out, follow-up MRI showed no recurrence of the tumor 41 months after the operation.
神经节神经胶质瘤是一种罕见的中枢神经系统肿瘤，通常包含肿瘤性神经节细胞和星形胶质细胞成分。迄今为止，仅有少数报告称含有少突胶质细胞组织的神经节神经胶质瘤病例。我们报告了一例 40 岁女性的神经节神经胶质瘤，主要由少突胶质细胞成分组成。磁共振成像显示右顶叶有一个界限清楚的囊性病变，伴有轻微的周围水肿。Gd-DTPA 造影剂给药后囊壁无强化。肿瘤被完全切除。组织学检查显示两种截然不同的成分混合存在：少突胶质细胞瘤和发育不良的神经节。第一个成分是弥漫性增殖的细胞，具有圆形核和核周晕；第二个成分显示明显的核仁，嗜碱性细胞质中含有尼氏体。少突胶质细胞成分的免疫组化研究对 OLIG 2 和 NKX2.2 呈阳性，但对突触素呈阴性。此外，通过 FISH 检测到 1p/19q 的 LOH。尽管未进行辅助治疗，但术后 41 个月的随访 MRI 显示肿瘤无复发。
