中枢神经系统血管周细胞瘤临床病理学观察

Clinicopathological features of haemangiopericytoma in central nervous system

目的探讨中枢性血管周细胞瘤(haemangiopericytoma,HPC)的临床特点、影像学特征、免疫表型。方法对4例中枢性HPC患者临床病理资料进行总结分析,随访3～24个月,并复习相关文献。结果男性2例,女性2例,平均年龄50岁。4例患者均先行肿瘤切除术,其中2例术后行放疗。免疫表型:3例vimentin、CD34、CD99均(+),3例EMA(-)性,1例EMA灶状(+)。例3和例4的Ki-67增殖指数分别为灶状5%和10%。随访3～24个月,肿瘤无复发及颅外转移。结论中枢性HPC发病率极低,其影像学表现与脑膜瘤相似,确诊均需免疫组化标记来证实,但其与脑膜瘤在生物学行为、组织学及治疗预后方面均不同,故中枢性HPC患者术后需进行长期随访,及早发现复发或转移并给予及时处理,可降低患者病死率。

Purpose To investigate the clinical features, imaging features and immunohistochemical phenotype of central nervous sys- tem haemangiopericytoma （HPC）. Methods The medical records were analyzed in four cases of patients with central HPC, with fol- low-up for 3 to 24 months, and review of the literature. Results There were 2 males and 2 females in this group, with average age of 50 years. All the tumors were surgically removed firstly, and 2 cases of them received postoperative radiation therapy. Immunophentyp- ing showed that vimentin, CD34 and CD99 were positive in 3 cases and EMA negative in 1 case, focally positive in 1 case. Case 3 and case 4 showed Ki-67 labelling index was 5% and 10% , respectively. Four cases were followed up for 3 -24 months, without tumor re- currence and extracranial metastasis. Conclusions Central HPC incidence is very low, and its imaging similar to meningioma. The diagnosis is confimled by pathology and immunohistoehemistry. But it is different from meningioma in the biological behaviors, histolo- gy and prognosis, and therefore patients with central HPC need a long-term follow-up after surgery for early detection of recurrence or metastasis and timely treatment.