中枢神经系统血管周细胞瘤的临床特点及治疗  

Clinical characteristics and treatment of intracranial hemangiopericytomas

作　　者：徐波涛[1] 漆松涛[1] 王洪筱[1] 吕超[1] 张喜安[1] 彭林[2] 

机构地区：[1]南方医科大学南方医院神经外科,广州510515 [2]南方医科大学南方医院惠侨科,广州510515

出　　处：《中华神经外科杂志》2014年第8期792-795,共4页Chinese Journal of Neurosurgery

基　　金：国家临床重点专科建设项目

摘　　要：目的 回顾总结中枢神经系统血管周细胞瘤（ⅢPCs）临床表现、影像特点、治疗及预后,优化诊疗流程.方法 回顾10例HPCs病例资料,统计其临床特点、影像学表现、治疗经过、病理及预后.结果 初发时幕上镰旁4例,中颅底和颅后窝各3例.肿瘤多跨大脑镰或邻近静脉窦生长,内部和周边无钙化,局部颅骨可有侵蚀但无增厚,部分肿瘤分叶状,蒂较窄或缺少脑膜尾征.首次均实施手术,6例全切,4例次全切.术后病理WHOⅡ级7例,Ⅲ级3例,复发者术后均为Ⅲ级.所有患者术后接受放疗,1例化疗.随访时间13 ～ 137个月,平均62.5个月,2例复发.复发病灶再次手术切除,转移灶给予局部放疗,效果满意.结论 虽然HⅡCs极少见、易误诊,但其临床和影像学仍有一些特点;手术全切是治疗首选,术后应积极辅助放疗;手术切除和放射外科治疗可以反复使用以控制复发或转移肿瘤.Objective To retrospectively summarize the clinical manifestations,treatment and prognosis of intracranial hemangiopericytomas（HPCs）,and to optimize process of diagnosis and treatment.Methods The data of 10 cases of HPCs were reviewed,including their clinical characteristics,imaging display,treatment process,pathology and prognosis.Results The onset lesions included 4 cases in supratentorial falx,3 in middle cranial fcssa,3 in posterior cranial fossa.The tumors grew across the cerebral falx or adjacent venous sinus.The imaging examinations showed that there was no calcification both inside and on margin of the lesions.The adjacent skull could be eroded but there was no thickening.Part of phyllodes tumors were found to be with narrow necks and lack of dural tail signs.All the patients were performed operation,6 cases achieved total removal and 4 subtotal.The pathological examinations after operation showed 7 cases of WHO Ⅱ and 3 WHO Ⅲ.All the recurring cases were WHO Ⅲ in both onset and recurrence lesions.All the cases accepted radiation therapy,and 1 recurring case was given chemotherapy after the removal of relapse lesion.The follow-up period was from 13 months to 137 months （mean duration：62.5 months）.There were 2 recurred cases with second surgery and local radiation for metastasis.Conclusions Although HPCs are extremely rare and easily misdiagncsed as meningiomas,there were still some characteristic features in clinical manifestation and imagings.The adjuvant radiotherapy should be given after operation.When the tumor recurrence was confirmed,surgery and radiosurgery,which could be performed repeatedly,should be recommended in order to control the recurrence or metastasis of tumors.

关 键 词：血管周细胞瘤 中枢神经系统 症状 手术 放疗 预后 

分 类 号：R739.4[医药卫生—肿瘤]