Vuia O
Eur Neurol. 1977;16(1-6):1-10. doi: 10.1159/000114873.
Two cases of malignant tumours of the nerve sheaths are reported. In the first patient, who had von Recklinghausen's disease, light- and electron-microscopic investigations showed an undifferentiated mesenchymal tumour of the radial nerve with the property of producing collagen fibres, an aspect characteristic of sarcoma of the fibroblastic type. In the second case a pleomorphic cell sarcoma of the cranial nerves of the left cerebellopontine angle was present. The morphologic aspects of these tumours show them to be primary mesenchymal tumours of the nerve (neurofibrosarcoma, neurogenic sarcoma) originating in the fibroblastic-like elements of the nerve sheaths. The clinical and nosologic framework of this type of malignant tumour of the nerve sheaths is discussed.
报告了两例神经鞘恶性肿瘤。第一例患者患有冯·雷克林豪森病，光镜和电镜检查显示桡神经有未分化间充质肿瘤，具有产生胶原纤维的特性，这是成纤维细胞型肉瘤的特征表现。第二例为左侧小脑脑桥角脑神经的多形性细胞肉瘤。这些肿瘤的形态学表现表明它们是起源于神经鞘成纤维样成分的原发性神经间充质肿瘤（神经纤维肉瘤、神经源性肉瘤）。本文讨论了这种类型神经鞘恶性肿瘤的临床和疾病分类框架。
