Kikuchi K, Saito M
Department of Neurosurgery, Akita University School of Medicine, Honjo, Japan.
Tohoku J Exp Med. 1999 Jul;188(3):245-56. doi: 10.1620/tjem.188.245.
A case of an unusual spinal neuronal tumor is described in a 36-year-old woman presenting with a buttock pain. The spinal tumor was fully characterized by neuroradiological means, and in particular MRI was of significant value in delineating the extension of the tumor within the spinal canal and its exophitic growth pattern. Pathologically, a well circumscribed tumor originating from the intradural filum terminale characteristically comprised both large and small cells, resembling mature and immature neuronal cells, respectively. In addition, two neuronal markers, i.e., chromogranin A (CGA) and neuron-specific enolase (NSE), and other markers such as glial fibrilary acidic protein (GFAP), S-100 protein, HNK-1, tyrosine hydroxylase and beta 2-microgloblin were investigated immunohistochemically. We found that both neuronal cells expressed immunoreactivity for CGA and NSE, and small neuronal cells showed more intense CGA immunoreactivity, indicating an earlier stage of neuronal differentiation. Weakly positive immunoreactivity for HNK-1 was also demonstrated in small neuronal cells, consistent with evidence of maturation along a neuronal differentiation. From these findings a pathological diagnosis of ganglioneuroma was made. This unique group of ganglion-cell spinal tumors is reviewed in the literature and differential diagnosis and immunohistochemical features are discussed.
本文描述了一例36岁女性的罕见脊髓神经元肿瘤病例，该患者表现为臀部疼痛。通过神经放射学手段对脊髓肿瘤进行了全面特征描述，尤其是MRI在描绘肿瘤在椎管内的延伸及其外生性生长模式方面具有重要价值。病理上，一个起源于硬脊膜内终丝的边界清晰的肿瘤特征性地包含大细胞和小细胞，分别类似于成熟和未成熟的神经元细胞。此外，免疫组织化学研究了两种神经元标志物，即嗜铬粒蛋白A（CGA）和神经元特异性烯醇化酶（NSE），以及其他标志物，如胶质纤维酸性蛋白（GFAP）、S-100蛋白、HNK-1、酪氨酸羟化酶和β2-微球蛋白。我们发现两种神经元细胞均对CGA和NSE呈免疫反应性，小神经元细胞显示出更强的CGA免疫反应性，表明神经元分化处于早期阶段。小神经元细胞中也显示出对HNK-1的弱阳性免疫反应性，这与沿神经元分化成熟的证据一致。基于这些发现，做出了神经节神经瘤的病理诊断。本文对这一独特的神经节细胞脊髓肿瘤组进行了文献综述，并讨论了鉴别诊断和免疫组织化学特征。
