Tsukahara Y, Sato M, Kato J, Hirabayashi T, Fukuta T
Nihon Sanka Fujinka Gakkai Zasshi. 1985 Sep;37(9):1915-8.
This paper reports a malignant primary nerve sheath tumor (MPNST) which originated in the obturator nerve in a 54-year-old woman. That the tumor originated in the nerve sheath is supported by the site of occurrence, which was consistent with the obturator nerve, an arrangement of cells similar to that in schwannoma, and that extracellular basement membrane was noted ultrastructurally. This tumor was classified not as malignant schwannoma but as a nerve sheath fibrosarcoma because the matrix of tumor tissues contained abundant mucinous materials, some tumor cells were fibroblastic, and the clinical change was rapid. In general, with plexiform neurofibroma there is a familial manifestation and it occurs as a complication of von Recklinghausen's disease, associations not demonstrated in this patient. From these findings, this tumor is considered to be an extremely rare nerve sheath fibrosarcoma which originated from a malignant change in a sporadic and solitary plexiform neurofibroma.
本文报道了一例起源于闭孔神经的恶性原发性神经鞘瘤（MPNST），患者为一名54岁女性。肿瘤起源于神经鞘这一点得到了发病部位的支持，该部位与闭孔神经一致，细胞排列类似于神经鞘瘤，并且在超微结构上观察到细胞外基底膜。该肿瘤未被归类为恶性神经鞘瘤，而是被归类为神经鞘纤维肉瘤，因为肿瘤组织基质中含有丰富的黏液物质，一些肿瘤细胞呈成纤维细胞样，且临床变化迅速。一般来说，丛状神经纤维瘤有家族性表现，是冯·雷克林豪森病的并发症，而该患者未表现出这些关联。基于这些发现，该肿瘤被认为是一种极其罕见的神经鞘纤维肉瘤，起源于散发性孤立性丛状神经纤维瘤的恶性变。
