恶性周围神经鞘膜瘤26例临床病理分析

Clinical and pathological analysis of 26 malignant peripheral nerve sheath tumor cases

目的分析恶性周围神经鞘膜瘤的临床及病理特点。方法 26例恶性周围神经鞘膜瘤患者,光镜下观察切片的组织学类型和免疫表型并进行临床病理分析。结果 26例恶性周围神经鞘膜瘤患者中,多数患者以肿块为临床症状,多发生于躯干、背部、四肢和颈部,年龄26~59岁,平均年龄（42.5±5.6）岁;组织学形态类型多样,免疫组化中Vimentin一抗抗体26例全为阳性,S-100、CK、EMA、NSE、Desmin、Myoglobin、Ki-67一抗抗体呈部分阳性,SMA、CD117、CD34一抗抗体26例样本均为阴性。结论由于恶性神经鞘膜瘤的组织学形态多样,鉴别诊断较为困难,临床采用免疫标记的方法结合肿瘤发生部位来进行恶性周围神经鞘膜瘤的诊断。

Objective To analyze clinical and pathological features of malignant peripheral nerve sheath tumor. Methods Histological type and immunophenotyping of 26 patients with malignant peripheral nerve sheath tumor were observed by section under light microscope for clinical and pathological analysis. Results The majority of patients among the 26 cases had lumps as clinical symptom, mainly in trunk, back, extremities and neck. They aged in ranges of 26~59 years old, with mean age as（42.5±2.6） years old. Their histological types were various. Immunohistochemistry showed all 26 positive cases with Vimentin-antiantibody, partial positive cases with S-100, CK, EMA, NSE, Desmin, Myoglobin, Ki-67-antiantibody, and all 26 negative cases with SMA, CD117, CD34-antiantibody. Conclusion Differential diagnosis of malignant peripheral nerve sheath tumor is difficult due to its various histological patterns. Therefore combination of immunolabelling and tumor site is used in clinical diagnosis of malignant peripheral nerve sheath tumor.