发生颅外转移的脑膜血管周细胞瘤临床病理观察

Meningeal haemangiopericytoma(M-HPC) with extracranial metastasis:a clinicopathologic study

目的探讨脑膜血管周细胞瘤发生颅外转移的临床病理特征、诊断与鉴别诊断要点。方法对9例发生颅外转移的脑膜血管周细胞瘤进行临床病理分析、组织形态学及免疫组化染色观察，结合文献对其，临床表现、病理形态特点及鉴别诊断进行探讨。结果患者年龄32～59岁，男性3例，女性6例。转移部位有肝、肺、骨、腹腔、皮下等。转移间隔时间0．5～10年不等，光镜下瘤细胞为形态大小较一致的短梭形细胞，瘤细胞间富于小血管，呈裂隙状或“鹿角状”，核卵圆形，具有非典型性，核分裂象多少不等，WHOII级或III级。免疫组化瘤细胞波形蛋白均呈强阳性表达，CD34阳性程度不等，弥漫阳性或局灶阳性。结论脑膜血管周细胞瘤临床呈侵袭性，易复发和转移到中枢神经系统外，鉴别诊断包括孤立性纤维性肿瘤、脑膜瘤及间叶性软骨肉瘤等。

Objective To investigate the clinicopathological and diagnostic characteristics of meningeal haemangiopericytoma （M-HPC） with extracranial metastasis. Methods The clinical presentation, histo- logical changes and immunohistochemieal staining were analyzed in nine cases of M-HPC with extracranial metastasis and related literatures were reviewed. Results Their ages ranged from 32 to 59 years old, 3 males and 6 females. The neous tissue. The interval included liver, ranges from half sites of metastasis time of metastasis lung, bone, peritoneal cavity, and subcuta- a year to 10 years. Histologically, the tumor was composed of spindle-shaped cells and numerous vascular pattern with a ＂staghorn configuration＂. Im- munohistochemicalty, the cells were typically positive for vimentin, and CD34. Conclusions M-HPC is a rare malignant tumor with aggressive behavior and can easily recur or metastasize to extracranial sites Its differential diagnosis includes solitary fibrous tumor, meningioma, and mesenchymal chondrosarcoma