Pal L, Santosh V, Gayathri N, Das S, Das B S, Jayakumar P N, Shankar S K
Department of Neuropathology, NIMHANS, Bangalore, India.
Acta Neuropathol. 1998 Mar;95(3):318-23. doi: 10.1007/s004010050805.
An unusual case of cerebellar neurocytoma with rhabdomyomatous differentiation in a 6-year-old boy is reported. Immunocytochemical and ultrastructural features of the tumour were studied. Abortive synapse formation, the presence of clear vesicles and synaptophysin immunoreactivity of the tumour cells indicated its intermediate neuronal differentiation, while the presence of myoblasts and myotubes and immunolabelling by desmin confirmed the rhabdomyomatous differentiation. In addition, the mesenchymal cells variably expressed neurofilament protein and glial fibrillary acidic protein, suggesting inductive interaction between the neuroectodermal and ectomesenchymal elements and persistence of the pleuripotential nature of the cells along the rhombic area of the brain stem.
报告了一例6岁男孩患小脑神经细胞瘤并伴有横纹肌样分化的罕见病例。对该肿瘤的免疫细胞化学和超微结构特征进行了研究。肿瘤细胞中出现的不完全突触形成、清亮小泡的存在以及突触素免疫反应性表明其具有中间神经元分化，而成肌细胞和肌管的存在以及结蛋白免疫标记证实了横纹肌样分化。此外，间充质细胞可变地表达神经丝蛋白和胶质纤维酸性蛋白，提示神经外胚层和外间充质成分之间存在诱导性相互作用，且细胞沿脑干菱形区的多能性特征持续存在。
