45例恶性周围神经鞘膜瘤临床病理分析

A clinicopathologic study of 45 cases of malignant peripheral nerve sheath tumors

目的了解恶性周围神经鞘膜病（MPNST）的临床病理特点。方法分析45例MPNST的临床特点，光镜观察其病理组织学变化并采用免疫级化SP法研究其免疫表型的异质性。结果①本组病例年龄2-68岁，中位年龄41岁，常发生在四肢、躯干及脊柱等中轴部位；临床上最常见的症状为肿块。②MPNST的形态学瘤谱范围广，包括梭形细胞、上皮样细胞、原始神经上皮分化区及异源性成分等。③免疫组化染色除S-100阳性外，Vim、EMA、Keratin、Desmin及GFAP部分阳性，但SMA阴性。结论MPNST是一种形态学瘤谱范围广、免疫表型异质性大的恶性肿瘤，与神经纤维瘤病关系密切。

Objective To investigate the clinicopathologic characteristics of malignant peripheral nerve sheathtumor (MPNST). Methods Using strict clinical and pathologic criteria for the inclusion of cases, the clinico-pathologic features of 45 cases of MPNST were reviewed by microscope and immunohistochemistry SP method.ResuIts diThe patients age ranged from 2 to 68 years old. The majorlty of the tumors were axial in location;the rest were peripheral. Patients typically presented with a mass of variable duration; Tumors demonstrateda wide histolOgic spectrum that included spindled, epithelioid, and priemitive neurocpithelial-like cells as well asheterologOus elements. Immunohistochemical staining reveal ed Vim in 43 of 45 cases (9 5. 5 % ), EMA (16 of45 cases, or 35 .5 % ), CK(10 of 45 cases, or 22 .2% ) and Desmin (6 of 45 cases, or 13 .2% ). ConclusionMPNST is histolOgically and rather divergent, immu nohi stochemically fairly heterologous, showing close rela -tionship with Von Recklinghausen's disease.