Ducatman B S, Scheithauer B W
Cancer. 1984 Sep 15;54(6):1049-57. doi: 10.1002/1097-0142(19840915)54:6<1049::aid-cncr2820540620>3.0.co;2-1.
Malignant peripheral nerve sheath tumors are uncommon sarcomas of Schwann cell or fibroblastic derivation which occasionally show histologic evidence of focal divergent differentiation to rhabdomyosarcoma, osteosarcoma, chondrosarcoma, angiosarcoma, epithelial elements, or a combination thereof. The finding of these heterotopic elements in nerve sheath sarcomas is believed to illustrate the differentiating capacity of neuroectodermal tissue. The authors present the clinicopathologic features of 17 such tumors, which represent 14.7% of the malignant peripheral nerve sheath tumors in the authors' institutional experience. Most of the lesions were associated with von Recklinghausen's disease. These tumors do not seem to differ from ordinary malignant peripheral nerve sheath tumors in presentation, operative, gross, and microscopic features, response to therapy, or prognosis.
恶性外周神经鞘瘤是一种罕见的源自施万细胞或成纤维细胞的肉瘤，偶尔在组织学上显示出向横纹肌肉瘤、骨肉瘤、软骨肉瘤、血管肉瘤、上皮成分或它们的组合局灶性分化的证据。神经鞘肉瘤中这些异位成分的发现被认为说明了神经外胚层组织的分化能力。作者展示了17例此类肿瘤的临床病理特征，在作者所在机构的经验中，这些肿瘤占恶性外周神经鞘瘤的14.7%。大多数病变与冯·雷克林豪森病相关。这些肿瘤在临床表现、手术、大体和显微镜特征、对治疗的反应或预后方面似乎与普通恶性外周神经鞘瘤并无不同。
