Ishikura Toshiki, Hirono Seiichiro, Ota Masayuki, Yokoyama Daiki, Kobayashi Masayoshi, Matsutani Tomoo, Iwadate Yasuo
Department of Neurological Surgery, Chiba University Graduate School of Medicine, Chiba, Chiba, Japan.
Diagnostic Pathology, Chiba University Graduate School of Medicine, Chiba, Chiba, Japan.
NMC Case Rep J. 2024 Dec 25;11:427-433. doi: 10.2176/jns-nmc.2024-0189. eCollection 2024.
Ganglioglioma, a glioneuronal neoplasm, typically presents in adolescents' temporal lobes. While pediatric brainstem gangliogliomas (BSGGs) are well documented, adult BSGGs are limited, resulting in a lack of comprehensive understanding of their pathophysiology and prognosis. A 41-year-old woman who presented with dizziness and numbness in her right upper extremity and right face underwent radiological examination. A dorsally exophytic tumor in the medulla oblongata was identified. Moderate uptake of C-methionine on positron emission tomography suggests a low-grade glioma. Histopathological evaluation, following a suboccipital craniotomy and biopsy under neuromonitoring, confirmed the tumor as a mix of neoplastic ganglion cells and glial cells, which exhibited immunoreactivity for chromogranin A and OLIG2, respectively. Eosinophilic granular bodies and Rosenthal fibers were also observed. These findings confirmed the diagnosis of a ganglioglioma. The BRAF V600E mutation tested negative by real-time polymerase chain reaction. No postoperative adjuvant treatment was administered, and no progression of the residual tumor was noted 34 months post-surgery. Increased reporting of adult BSGGs, complete with detailed radiological, molecular, and genetic profiles, as well as their clinical course, is essential for clarifying our understanding of this rare entity's oncogenic pathway, optimal management strategy, and prognosis.
神经节胶质瘤是一种神经胶质神经元肿瘤,通常发生于青少年的颞叶。虽然小儿脑干神经节胶质瘤(BSGGs)已有充分记录,但成人BSGGs的病例有限,导致对其病理生理学和预后缺乏全面了解。一名41岁女性因出现头晕及右上肢和右面部麻木而接受了放射学检查。检查发现延髓有一个向背侧外生性肿瘤。正电子发射断层扫描显示C-蛋氨酸摄取适度,提示为低级别胶质瘤。在神经监测下进行枕下开颅手术及活检后,组织病理学评估证实该肿瘤为肿瘤性神经节细胞和胶质细胞的混合体,分别对嗜铬粒蛋白A和OLIG2呈免疫反应性。还观察到嗜酸性颗粒小体和罗森塔尔纤维。这些发现证实了神经节胶质瘤的诊断。实时聚合酶链反应检测显示BRAF V600E突变呈阴性。术后未进行辅助治疗,术后34个月未发现残留肿瘤进展。增加对成人BSGGs的报告,包括详细的放射学、分子和基因特征以及临床病程,对于阐明我们对这种罕见疾病的致癌途径、最佳管理策略和预后的理解至关重要。