目的探讨低度恶性神经束膜瘤（LGMP）的临床病理学特点、免疫组化。方法对1例LGMP进行组织形态学观察、免疫组化染色、ALK基因检测,并随访及复习相关文献,进行综合分析。结果患者女性,58岁,左大腿肿物5个月,直径10 cm。镜下肿瘤细胞呈结节状,可见细胞疏松区和致密区。细胞疏松区：细胞梭形,部分细胞呈波浪样,细胞异型性小,核分裂象少见,间质黏液变性。细胞致密区：瘤细胞丰富,片状,漩涡状排列,细胞呈胖梭形,上皮样,异型性较大,可见核仁,核分裂象约5个/10HPF,肿瘤细胞浸润性生长,侵犯横纹肌组织,未见坏死。肿瘤局部见类似神经丛样结构。免疫组化：肿瘤细胞CD57、EMA、CD56、Claudin-1及GLUT-1（＋）,S-100、CK、Actin（SM）、CD34、h-caldesmon、CD99、NSE、GFAP（-）,Ki-67阳性指数为10%,ALK基因检测阴性。结论 LGMP是罕见的软组织肿瘤,诊断依赖于病理组织学、免疫组化,必要时电镜检查。治疗以手术完整切除为主,预后较好。
Objective To investigate the clinicopathological features and immunohistochemical findings of low-grade malignant perineurioma（ LGMP）. Methods The histopathological changes,immunophenotype and molecular findings,treatment and prognosis were evaluated in 1 case of LGMP with review of the relevant literatures. Results One female aged 58-year-old presented with a mass in left leg,with a diameter of 10 cm. Microscopically,in low power field,the tumor cells arranged in nodules with loose and dense areas. In loose area,the cells were fusiformis and part of those were wave-like,with elongated nuclei. Rare mitotic figures were observed,and stroma was mucoid degeneration. In dense area,the tumor cells were rich,with flaky,whirlpool arrangement,with obvious atypia. Mitotic figures were about 5/10 HPF.The tumor cells invaded into muscle tissue with an invasive growth pattern,and necrosis was not observed. Finally,in some area,there was locally neural plexus-like structure. Immunohistochemically,tumor cells were positive for CD57,EMA,CD56,Claudin-1 and GLUT-1,while S-100,CK,Actin（ SM）,CD34,h-caldesmon,CD99,NSE and GFAP were negative,and Ki-67 index was 10%. ALK gene detection was negative. Conclusion LGMP is a rarely soft tissue tumor,its diagnosis depends on pathological histology and immune phenotyping,and electron microscopic examination is important if necessary. Treatment is priority to completely surgery resection,with a better prognosis.
Chinese Journal of Diagnostic Pathology