肺原发性恶性外周神经鞘瘤的临床病理分析

Primary malignant peripheral nerve sheath tumour of the lung

目的 探讨肺脏原发性恶性外周神经鞘瘤 (MPNST)的临床病理学特征及诊断、鉴别诊断要点。方法 对 2例肺MPNST进行临床病理学分析及免疫组织化学与超微结构研究。结果 2例光镜下均显示MPNST的形态特点 ,免疫组织化学显示S 1 0 0蛋白、MBP、Vim、NSE肿瘤细胞呈阳性表达 ,电镜观察 1例可见特征性Luse小体。结论 肺原发性MPNST极为罕见 ,临床诊断较困难。根据其光镜病理形态特征 ,S 1 0 0蛋白、MBP免疫组织化学检测和 (或 )电镜检查可确诊。结合文献 ,该肿瘤早期无明显临床症状 ,一般发现时已属晚期 ,预后差。病理学上应与肺脏平滑肌肉瘤、纤维肉瘤、单相型滑膜肉瘤。

Purpose To investigate the clinical pathologic characteristics and to probe into the diagnosis and differential diagnosis of the primary malignant peripheral nerve sheath tumor(MPNST) of the lung. Methods Two cases of primary pulmonary MPNST were examined with HE sections, immunohistochemical stains and electron microscopy. Results Histological examination showed the characteristics of MPNST. The positive singals of S 100, MBP, Vim and NSE were detected in both cases. The Luse body was found with electron microscopy in one case. Conclusions Primary pulmonary MPNST is extremely rare. It is difficult for clinical doctor to make the diagnosis. The tumor has its pathological features, so a definite diagnosis can be made with the pathological examination and immunohistochemical stains for S 100, MBP, or ultrastructural observation. Because the patient has no special clinical symptoms, it is in its late stage when discovered. Primary pulmonary MPNST should be distinguished from leiomyosarcoma, fibrosarcoma, monophasic synovial sarcoma, and sarcomatoid carcinoma.