Bullock M J, Bedard Y C, Bell R S, Kandel R
Department of Pathology, Mount Sinai Hospital, Toronto, Ontario, Canada.
Arch Pathol Lab Med. 1995 Apr;119(4):367-70.
We report a case of a malignant peripheral nerve sheath tumor arising in the distal femur of a 28-year-old man who had no stigmata of von Recklinghausen's disease. Roentgenograms and computed tomographic scans of the distal femur showed a single lytic intraosseous lesion with minimal soft tissue extension. Light microscopy demonstrated a tumor composed of spindle cells with myxoid areas. There was some nuclear pleomorphism and mitotic activity. The tumor cells were immunoreactive for S100 protein and neuron-specific enolase. Ultrastructurally, there were cells with long processes, which were focally invested by basal lamina. Some cells had numerous micropinocytotic vesicles. The tumor recurred in the femur 15 months following the initial curettage. Computed tomographic scan of the lungs at that time showed a nodule, which has since enlarged. Primary osseous malignant peripheral nerve sheath tumors are uncommon. A literature review revealed only 18 previous cases, the majority of which occurred in the mandible or maxilla. This is a rare case of intraosseous malignant peripheral nerve sheath tumor of a long bone, with both immunohistochemical and ultrastructural confirmation of nerve sheath differentiation.
我们报告一例发生于一名28岁男性股骨远端的恶性外周神经鞘瘤，该患者无冯雷克林霍增氏病的体征。股骨远端的X线片和计算机断层扫描显示一个单一的溶骨性骨内病变，软组织延伸极少。光镜检查显示肿瘤由具有黏液样区域的梭形细胞组成。存在一些核多形性和有丝分裂活性。肿瘤细胞对S100蛋白和神经元特异性烯醇化酶呈免疫反应。超微结构上，有一些具有长突起的细胞，这些细胞局部被基膜包绕。一些细胞有大量微吞饮小泡。初次刮除术后15个月，肿瘤在股骨复发。当时的肺部计算机断层扫描显示一个结节，此后该结节增大。原发性骨恶性外周神经鞘瘤并不常见。文献回顾仅发现18例先前病例，其中大多数发生在下颌骨或上颌骨。这是一例罕见的长骨骨内恶性外周神经鞘瘤，具有免疫组织化学和超微结构证实的神经鞘分化。
