中枢神经系统血管周细胞瘤的临床病理分析

Clinicopathological and immunohistochemical analysis of meningeal hemangiopericytoma

目的探讨中枢神经系统血管周细胞瘤（HPC）的临床病理特征及其鉴别诊断。方法运用组织病理学和免疫组织化学对23例HPC进行观察并随访,结合文献对其临床特点、病理形态特点及鉴别诊断进行探讨。结果 HPC的临床症状主要为肿瘤压迫及颅内高压征,1例表现为颅内出血症状。镜下观察肿瘤细胞丰富,无特定的排列方式,瘤细胞形态一致,胞质界限不清,淡嗜酸性,胞核圆形或卵圆形,核仁不明显,核分裂像0-8个/10 hpf。间质有丰富的分枝状薄壁血管。免疫组化：Vimentin、CD34及CD99（＋）,Ki-67 1%-40%（＋）,部分病例E-Cadherin局部（＋）,CK、EMA、SMA、S-100、GFAP以及PR均（-）。网织纤维染色示单个瘤细胞被网状纤维包绕。结论 HPC是一种少见的中枢神经系统肿瘤,熟练掌握其形态学特点及免疫组化的运用有助于其诊断和鉴别诊断。肿瘤容易复发和转移,术后需长期随访。

【Objective】 To study the clinicopathologic features of meningeal hemangiopericytoma（HPC） and its differential diagnosis.【Methods】 23 cases of meningeal HPC were studied by light microscopy and immunohistochemistry.The clinical features and follow-up information were analyzed.【Results】 The clinic characters mainly manifested as intracranial hypertension and oncothlipsis,one case presented with intracranial hemorrhage.Histologically,the tumors were high cellularity with ＂patternless pattern＂.They has similarly spindle shape and contained eosinophilic cytoplasm,with round or oval nuclei,indistinct nucleoli and 0～8/10 hpf of mitotic figures.Immunohistochemical study showed that the tumor cells were positive for Vimentin,CD34 and CD99,10 cases locally were reacted to E-cadherin,but were negtive to CK,EMA,SMA,S-100 protein,PR and GFAP.Ki-67 indexes were 1%～40%.Reticular fiber staining displayed that single tumor cell was surrounded by reticular fiber.【Conclusion】 HPC is a rare tumor entity of central nervous system.To be familiar with its characteristics and appropriate application of markers,such as CD34 and EMA,would be helpful for arriving at a correct diagnosis.The tumor is apt to recur and extracranial metastasize,so that long time follow-up is necessary.