颅内血管周细胞瘤的临床病理分析

Clinicopathological features of intracranial hemangiopericytoma

目的探讨颅内血管周细胞瘤（HPC）的临床病理特征、免疫表达、诊断及鉴别诊断。方法对7例颅内HPC患者的临床病理资料，结合相关文献分析其临床表现、病理特点。结果7例患者中男4例，女3例，4例术后行放射治疗，其中2例复发；3例术后未作治疗，1例复发。光镜下见瘤组织均由密集的梭形细胞或卵圆形瘤细胞组成，其间有口径不等的鹿角状分支血管，肿瘤产生的网状纤维包绕瘤细胞。免疫组化：Vim、CD34、CD99及Bcl-2（7／7）强阳性；CD57（1／7）、EMA（2／7）及SMA（3／7）局灶阳性；Ki-67指数5％～10％，复发病例〉25％。随访期内，3例复发但无颅外转移。结论颅内HPC具有明显的侵袭行为，诊断时应结合临床、免疫组化及网状纤维染色排除其他具有相似组织形态肿瘤。

Objective To analyze the clinicopathological features, immunohistochemical phenotype, diagnosis and differential diag- nosis of intracranial hemangiopericytoma （HPC）. Methods A retrospective study was conducted on seven cases of HPC. Clinical manifestations, pathological characteristics and differential diagnosis were analyzed, and related literature was reviewed. Results Among the 7 HPC patients, 4 were male and 3 were female. Moreover, 4 patients underwent postoperative radiotherapy and recur- rence was observed in two of them; of the 3 patients who hadn＇ t received any treatment other than surgery, 1 suffered from recur- rence. For pathological findings, spindle cells or ovoid tumor cells were densely arranged in HPC tissue. Antler branch blood ves- sels with different diameters were found in tumor tissue. As for immunohistochemistry, Vim, CD34, CD99 and Bcl-2 were stained strongly positive （7/7） ; CD57 （1/7） , EMA （1/7） and SMA （3/7） were focally positive; Ki-67 index was 5% - 10% and more than 25% in recmxent cases. For a 5-year follow up, 3 cases of recurrence were observed, without extracranial metastasis. Conclusion HPC is invasive. The diagnosis should be made on the basis of clinical diagnosis, immunohistochemical findings and reticular fiber staining. Differential diagnosis included other tumors with similar cell arrangements.