颅内血管外皮细胞瘤18例临床诊断与治疗分析

目的探讨颅内血管外皮细胞瘤的诊断与治疗。方法回顾性地对我院神经外科收治的颅内血管外皮细胞瘤18例患者的临床特点、诊断、治疗和预后进行分析。结果 18例患者中,男女比例为1.57∶1,平均年龄46.3岁。主要临床表现为头痛(77.8%)及局部神经系统症状。术前所有病例均未确诊,术中冰冻切片获得倾向性诊断,细胞学及免疫组化确诊。所有肿瘤均为单发,最大径平均为5.6 cm。免疫组化CD34阳性率为88.9%,波形蛋白(Vim)除1例不确定外,余均为阳性,一半的病例增殖细胞核抗原(Ki-67)为阳性,上皮膜抗原(EMA)均为阴性。全切10例(55.6%),次全切8例(46.6%),12例(66.7%)术后1个月内辅以立体定向γ-刀放射治疗。术后均获随访,随访期为1.5～10年。至今12例原位复发,无远处转移。结论颅内血管外皮细胞瘤术前诊断相当困难,目前主要依赖病理学检查,治疗上应旨在手术全切,术后辅以放疗。     

伽玛刀治疗脑膜血管外膜细胞瘤的临床分析

目的 探讨伽玛刀治疗在脑膜血管外膜细胞瘤的综合治疗中的临床作用.方法 回顾性分析1994年12月至2006年12月于本中心行伽玛刀治疗并随访的22例术后复发的患者,男13例,女9例;58个瘤灶,平均体积5.4 cm3,平均周边剂量13.5 Gy,平均中心剂量28.2 Gy.结果 平均随访期26个月.22例中,颅内转移7例,颅外转移3例,死亡4例,平均生存期67.7个月;58个瘤灶中,影像随访证实25个基本消失,13个明显萎缩,14个体积变化不明显,6个体积增大,肿瘤总控制率89.7%.结论 伽玛刀治疗对术后残留或复发的中小型脑膜血管外膜细胞瘤安全有效,改变了以往综合治疗的策略.     

颅内血管外皮细胞瘤诊断和治疗

目的探讨分析颅内血管外皮瘤的诊断、治疗及预后。方法对1997-2006年间收治的18例颅内血管外皮细胞瘤就其临床和影像学表现、病理学特点、治疗及随访结果进行回顾性分析。结果18例病人共行手术23次,全切10例,次全切9例,部分切除4例。术后辅助传统放疗6例,辅助γ-刀治疗3例。15例原发病例中5例复发,平均复发时间76.75个月。发生颅内转移2例,发生神经系统外转移2例。结论颅内血管外皮细胞瘤不同于脑膜瘤,有较高的局部复发率,并可以发生神经轴和神经系统外转移,争取手术全切 剂量不少于50Gy的辅助放疗应作为常规的治疗方案。对于颅内血管外皮细胞瘤病人需终生随访。     

中枢神经系统复发性血管外膜细胞瘤临床特点分析

目的探讨中枢神经系统复发性血管外膜细胞瘤(HPC)的临床特点。方法回顾性分析46例复发性HPC病人的临床资料,均行手术全切除或次全切除肿瘤,其中辅助放疗29例,辅助伽玛刀治疗7例。采用统计学分析复发性HPC病人术后的生存期及生存期的影响因素。结果 46例病人术后随访3~164个月,平均生存期为(41.6±4.4)个月,术后1、2、3、4年生存率分别为80.4%、65.2%、59.2%和53.8%。所有复发性HPC病人中,复发1次30例,其平均生存期为(36.9±4.1)个月;复发2次及以上16例,其平均生存期为(39.7±7.0)个月。随访期间死亡18例,其平均生存期为(14.2±5.6)个月;存活28例,其平均KPS评分为(86.8±14.2)分。经统计学分析:复发性HPC病人的生存期与性别及肿瘤部位、大小、质地、病理分级之间无相关性(P0.05);复发1次与复发2次及以上病人的生存期间亦无显著差异(P0.05)。结论积极综合治疗复发2次及以上的HPC病人,预后仍可令人满意。     

颅内血管外皮细胞瘤的诊断及治疗(附14例分析)

目的总结颅内血管外皮细胞瘤(HPC)的诊治经验。方法回顾性分析14例颅内HPC病人的临床资料和影像学表现。结果肿瘤全切除11例,次全切除3例。随访13例,随访时间5～62个月,恢复良好10例,死亡3例。颅内HPC的CT影像特点为平扫显示肿瘤呈等、高密度,未见瘤内钙化、邻近颅骨骨质增生及脑膜尾征等常见征象;MRI影像特点为T1WI显示肿瘤呈等、低混杂信号,T2WI显示肿瘤呈等或等、高混杂信号;增强后所有肿瘤明显强化,肿瘤多呈分叶状,窄基底附着硬膜,有丰富的血管流空,无脑膜尾征,无颅骨增生和钙化。结论颅内HPC具有比较典型的CT和MRI影像学特点,有助于临床诊断。颅内HPC首选手术治疗,术后可辅以放射治疗。     

血管外膜细胞瘤p53和Ki-67的表达与肿瘤分级和复发的关系

目的 探讨中枢神经系统血管外膜细胞瘤(HPC)中p53及Ki-67的表达与肿瘤的分级和复发的关系. 方法 回顾性分析北京天坛医院自2005年1月采用至2008年1月手术切除治疗的97例中枢神经系统HPC患者的临床资料,HE染色97例FIPC标本并按照WHO标准进行分级;免疫组织化学染色检测并比较不同分级、复发和未复发肿瘤标本中p53和Ki-67的表达.结果 HE染色检测显示HPC分级Ⅱ级74例,Ⅲ级23例;随访结果显示未复发HPC 49例,复发39例;免疫组化检测结果显示Ⅱ级HPC中p53和Ki-67阳性细胞数均明显低于Ⅲ级,未复发病例的Ki-67和p53阳性细胞数均明显低于复发病例,差异均有统计学意义(P<0.05).结论 Ki-67、p53的表达越高,HPC的组织分级越高,复发风险越高,可作为判断HPC患者预后的指标.     

颅内实质性血管母细胞瘤的诊断与治疗

近年来,我科共收治5例实质性血管母细胞瘤,经手术病理证实,取得满意的疗效,现报道如下.     

颅内血管外皮瘤l例报告

血管外皮瘤是一种很少见的血管源性肿瘤 ,起源于血管外皮 ,主要发生于肌肉和骨骼系统及皮肤 ,而发生于颅内者极为少见 ,现报告 1例如下。1　病例　男 ,5 6岁。因行走不稳伴头昏 4个月余于 2 0 0 2年10月 10日入院。患者入院前 4个月无明显原因出现头昏及走路不稳 ,无头痛 ,偶有呕吐。查体 :神志清楚 ,蹒跚步态 ,左侧咽反射减弱 ,悬雍垂偏向右侧 ,伸舌居中 ,双侧胸锁乳突肌和斜方肌对称 ,转颈及耸肩正常 ,颈软 ,四肢肌力正常 ,左侧上肢肌张力减弱 ,腱反射 (+) ,左侧指鼻试验及跟膝胫试验不准 ,闭目难立征阳性。头颅MRI显示左侧小脑半球有一…     

颅内血管外皮细胞瘤

血管外皮细胞瘤是一种软组织血管肿瘤,发生于颅内者较少见,以前将其归类于脑膜瘤,占脑膜瘤的1%.但近年来的研究证实它与脑膜瘤在组织学和生物学行为上均不相同,有较高的复发率,手术和放射治疗是主要处理方法,预后较脑膜瘤为差.本文参阅有关文献,就病理、电镜、临床表现、鉴别诊断、治疗及预后做一简要综述.     

脑脊膜血管外皮细胞瘤的诊断和治疗

目的探讨脑脊膜血管外皮细胞瘤诊断和治疗。方法回顾分析1984-2005年收治的32例脑脊膜血管外皮瘤,探讨其临床病理特征、临床治疗方法及预后。结果本组男女之比1.4:1;术前仅3例影像学诊断血管外皮瘤,其余均诊断为脑膜瘤。术前影像学误诊率为92%。所有病例均行手术治疗 放射治疗,27例影像学全切,4例次全切除,1例部分切除。获得随访的29例患者1年肿瘤复发率57%,所有病例免疫组化病理结果显示vimentin 。结论脑脊膜的血管外皮瘤为起源于脑脊膜问叶组织、低度恶性的肿瘤,男性多见,术前易与脑膜瘤相混淆,免疫组化病理有特征性表现;该肿瘤易复发,手术力争全切是改善预后的最重要因素;不管术中是否全切肿瘤,术后都应辅助放射治疗以改善预后。     

颅内血管外皮细胞瘤的临床特点及治疗

目的 总结临床病理确诊的25例颅内血管外皮瘤的临床特点及诊治体会.方法 回顾性分析资料完整的25例颅内血管外皮瘤的临床表现、影像学特点、病理、手术与随访结果.结果 颅内血管外皮瘤以中年男性多见,头痛及视乳头水肿明显,病情进展迅速.病理上本组25例中13例于每5个高倍视野见2个以上核分裂像.9例有片状坏死或小灶性出血.复发率高达36%(全切率84%).结论 颅内血管外皮细胞瘤是一种不常见的软组织肿瘤,具有与普通脑膜瘤不同的组织学与生物学行为,预后较差.     

Management of recurrent intracranial hemangiopericytoma

Intracranial hemangiopericytoma (HPC) is an aggressive meningothelial neoplasm. A particularly challenging aspect of management of patients with HPC is optimizing treatment for recurrence, progression, and extracranial metastasis. Here we describe a modern cohort of patients with recurrent HPC to better understand treatment strategies that may improve outcome. Patients managed at UCSF for recurrent intracranial HPC were compiled into a single database based on a retrospective review of patient records, including operative, radiologic, and clinic reports. Cox regression was performed to determine factors that independently predicted treatment outcomes. At UCSF, 14 patients with available treatment and follow-up data were seen for management of HPC recurrence. Eight patients underwent repeat surgical resection, of whom four received adjuvant external beam radiotherapy (EBRT), one received additional Gamma Knife radiosurgery (GKS), and one received brachytherapy. Radiosurgical intervention alone was utilized for recurrence in six patients, with four receiving GKS and two receiving CyberKnife. Nine patients suffered a second recurrence at a median time of 3.5 years following reintervention. Nine patients died following reintervention, with a median survival of 7.9 years following intervention for recurrence. In univariate analysis, factors associated with increased time to second recurrence included non-posterior fossa location (log rank, p < 0.05) and surgical resection with adjuvant EBRT (log rank, p < 0.05). The addition of adjuvant EBRT to surgical resection similarly extended overall survival compared to surgical resection alone (log rank, p < 0.05). GKS was associated with earlier second recurrence compared to surgically based strategies (log rank, p < 0.05). We conclude that when combined with surgical resection, EBRT appears promising in the extension of second recurrence-free survival and overall survival. This multimodality approach also appears to outperform GKS in extending time to second recurrence. Accordingly, when safe and feasible, surgical resection of recurrent HPC with adjuvant EBRT should be the first steps in management.     

Meningioma and intracranial hemangiopericytoma

Summary Electron microscopic study of 2 intracranial hemangiopericytomas and 7 meningiomas revealed fundamental morphologic differences between the 2 neoplasms. The most significant finding in hemangiopericytoma was the presence of ultrastructure features suggesting leiomyoblastic differentiation. These included characteristic fusiform intracytoplasmic and submembranous dense bodies, abundant cytoplasmic filaments, elongated cells with blunt-ended nuclei and juxtanuclear polarization of organelles. This observation is considered highly significant as an indicator of the pericytic nature of this tumor. In addition, hemangiopericytoma cells sometimes were arranged in spirals around pools of basement membrane-like material, perhaps a manifestation of the biologic capability of the cells to synthetize such material. Meningioma cells displayed as their main feature an ability to produce surface membrane specializations including interdigitations, desmosomes, zonulae adhaerentes and gap junctions. Sometimes the last 3 elements were linearly juxtaposed forming junctional complexes similar to those seen in certain epithelia. It is suggested that the characteristic whorls of meningioma are the result of cell interconnections arising from the specialized junctional attachments. Thus the distinctive morphology of the 2 neoplasms appears to derive from basic biologic properties of their elements.     

颅内黑色素瘤的诊断和治疗

目的 讨论颅内黑色素瘤的临床特点、影像学特征及治疗方案.方法 回顾性分析11例经证实的颅内黑色素瘤患者的资料,分析其临床表现、影像学特征,并对其临床特点和治疗方案进行讨论.结果 头部CT及MRI多可见瘤周有点状或条索状卫星灶.典型的黑色素瘤MRI表现为T1WI高信号、T2WI低信号,该表现具有特异性.11例手术中,4例全切,6例近全切,1例多发性转移瘤部分切除.术后患者多在3-12个月内死亡,最长不超过2年,平均生存期不到6个月.结论 颅内黑色素瘤发病率低,误诊率高,治疗以手术切除为主,放化疗及免疫疗法仅可使部分患者受益,预后极差.     

中枢神经系统血管周细胞瘤诊治分析

目的：探讨中枢神经系统血管周细胞瘤的临床特点及其治疗方法。方法回顾分析14例中枢神经系统血管周细胞瘤患者的临床资料,随访5～100个月,并进行文献复习。结果14例患者均行手术,术后病理明确诊断。肿瘤全切除8例,大部切除3例,部分切除3例。肿瘤复发7例（50％,4例为全切除,1例大部切除,2例为部分切除,术后均未曾放疗）,中位复发时间39个月,全切术后中位复发时间41．5个月,未全切除中位复发时间17个月。复发后再次手术及术后放疗仍可获得较长时间生存。结论血管周细胞瘤诊断依赖于病理,目前手术及术后放疗是较好的治疗办法,手术切除的完整性尤为重要。     

Diffusion weighted imaging may help differentiate intracranial hemangiopericytoma from meningioma

Background and purposeHemangiopericytoma and meningioma appear similar on routine diagnostic imaging and hence are difficult to distinguish. The purpose of our study was to examine the diffusion weighted imaging (DWI) characteristics of these two types of tumours.MethodsIn a retrospective study, each patient with hemangiopericytoma was matched with two meningioma patients based on tumour location and size. Minimum and mean apparent diffusion coefficients (ADC) were measured in the tumour and the contralateral normal-appearing white matter (NAWM). A normalized ADC was calculated. The two tumour types were subjectively assessed for heterogeneity on ADC maps.ResultsOf the 14 patients with histopathological proven hemangiopericytoma, only 7 had available DWI for analysis. These 7 patients were matched based on tumour location and size with 14 patients out of the 209 meningioma patients screened. Hemangiopericytomas were more heterogeneous on ADC maps (P < 0.001) and had a higher mean ADC compared to that of meningiomas (P < 0.001).ConclusionHemangiopericytomas showed heterogeneity on DWI and significantly higher ADC compared to that of meningiomas in our small study. These observations need to be confirmed in future studies with larger sample sizes.     

颅内血管外皮细胞瘤多器官转移临床分析

目的分析颅内血管外皮细胞瘤颅外多器官转移的临床特点,以提高诊治水平。方法回顾分析1例颅内血管外皮细胞瘤8a间的回访资料,根据临床表现、影像学表现、手术治疗及放射治疗效果及转移情况,结合文献探讨其临床特点。结果颅内血管外皮细胞瘤患者术前影像与脑膜瘤不易区别,导致误诊。术中见脑膜有明显侵犯,血供丰富。术后病理需免疫组化确诊。术后随访期内发生原位复发及颅内附近转移和颅外多发转移。结论颅内血管外皮细胞瘤发生颅外多发转移较为罕见,需与脑膜瘤、孤立性纤维瘤鉴别。治疗以手术切除为首选,术后应常规行放疗,复发时可再次放疗并需定期随访。     

颅内血管外皮细胞瘤

目的　对颅内血管外皮细胞瘤（ｈｅｍａｎｇｉｏｐｅｒｉｃｙｔｉｏｍａ , 简称 Ｈ Ｐ Ｃ） 其临床表现、辅助检查、病理、治疗及预后加强了解。方法　结合有关文献, 回顾我科近６ 年手术治疗的８ 例 Ｈ Ｐ Ｃ。结果肿瘤全切６ 例, 近全切２ 例, 术后随访４ 年６ 个月, ５ 例复发, 其中２ 例死亡。结论　颅内 Ｈ Ｐ Ｃ 预后不良, 即使全切局部复发率也很高, 放、化疗对残存或转移的肿瘤可能有短期缓解。