股骨伴上皮样分化的恶性外周神经鞘瘤临床病理观察

Malignant intraosseous peripheral nerve sheath tumour of femur: a clinicopathological analysis

目的探讨股骨恶性外周神经鞘瘤(MPNST)的临床病理学特征。方法对1例股骨MPNST的影像学情况、病理形态学特点、免疫组化及超微结构进行观察,并复习相关文献。结果 X线片显示股骨远端溶骨性破坏。镜下肿瘤由梭形细胞和上皮样细胞组成,伴地图状坏死和丰富的核分裂象。免疫组化肿瘤细胞vimentin、PGP9.5、S-100、CD56、CD57和p53(+)。电镜下瘤细胞胞质内见神经丝,细胞周围有不完整的基底膜。结论股骨MPNST罕见,诊断主要依据组织病理学特征、免疫组化和电镜特征,但分化差的肿瘤电镜结构对病理诊断意义不大。

Objective To review the clinicpathological features of intraosseous malignant peripheral nerve sheath tumour （MPNST） of the femur. Methods A new case of intraosseous MPNST of the femur was studied with histopathological, immunohistochemleal staining and ultrastructural observation together with the review of cases previously reported. Results Roentgenograms showed lytic destruction of the distal femur. Microscopically, the tumor consisted of spindle cells and epithelioid cells, accompanied by a map-like necrosis and high mitotic activity. Immunohistochenically, tumor cells were positive for Vim, S-100 and P53. Electron-microscopy revealed the neurofilament and incomplete basement membrane arrounding the tumor ceils. Conclusion Intraosseous MPNST of the femur is rare. The diagnosis is based on histopathological features, immunohistochemical staining and uhrastructure. But uhrastructure is of little help to poorly differentiated tumors.