脊髓血管母细胞瘤的显微外科治疗

目的 探讨脊髓血管母细胞瘤的显微外科治疗方法.方法 回顾性分析16例脊髓血管母细胞瘤病人的临床资料.术前均行血管造影,其中完全栓塞或部分栓塞9例.术中采用吲哚菁绿血管造影再次确认相关血管,采用显微外科手术切除肿瘤.结果 16例脊髓血管母细胞瘤均全切除.术后随访3个月,根据McCormick脊髓功能状态分级:明显改善12例,无变化3例,加重1例.结论 脊髓血管母细胞瘤通过显微外科手术切除可以取得良好疗效,术前血管造影栓塞和术中荧光血管造影有利于减少术中出血,提高手术安全性.     

脊髓室管膜瘤的显微外科治疗

目的探讨脊髓室管膜瘤显微外科治疗中的相关问题。方法回顾性分析22例脊髓室管膜瘤患者的临床资料。结果22例脊髓室管膜瘤中，肿瘤主体位于颈段12例，胸段7例，腰段3例。其中15例在肿瘤头端和，或尾端合并有脊髓空洞，囊性空洞呈分隔状5例，2例瘤体内有囊变。肿瘤全切15例，次全切除7例。根据治疗前后神经功能综合评价，好转14例，稳定7例，加重1例。结论对脊髓室管膜瘤应行积极的显微外科手术治疗，肿瘤能否全切与瘤体大小无明显关系，主要取决于肿瘤与脊髓的粘连程度；手术不能全切者术后辅以局部放疗可获得良好的疗效。     

脊髓血管母细胞瘤七例临床分析

目的总结脊髓血管母细胞瘤的治疗方法、疗效和临床经验。方法与结果 2012年2月至2017年2月共收治7例脊髓血管母细胞瘤患者,术前MRI显示脊髓占位明显、病灶周围血管流空影伴脊髓空洞症,病灶明显强化。经椎板切开行肿瘤切除术,手术全切除肿瘤(全切除5例、全切除责任病灶2例);术后组织病理证实为血管母细胞瘤。术后运动和感觉功能改善6例、无变化1例;McCormick分级Ⅰ级5例、Ⅱ级1例、Ⅲ级1例;无肿瘤复发病例。结论脊髓血管母细胞瘤为高度血管化的良性髓内肿瘤,可经手术全切除治愈。脊髓血流分布异常可能是导致临床症状的原因之一。     

脊髓髓内肿瘤显微外科治疗

目的总结脊髓髓内肿瘤的治疗经验。方法回顾性分析11例脊髓髓内肿瘤病人的临床资料,位于颈段2例,胸段6例,腰段3例。行MRI平扫和增强扫描明确诊断,均采用显微外科手术治疗,结合术中所见对诊断及治疗进行分析。结果肿瘤全切除7例,次全切除4例。术后症状明显改善8例,无改善2例,加重1例,无手术死亡。按McCormick脊髓功能状态分级标准:Ⅰ级8例,Ⅱ级1例,Ⅲ级1例,Ⅴ级1例。随访7例,时间6～24个月,复发2例。结论 MRI是诊断脊髓髓内肿瘤的首选方法,手术切除肿瘤能改善症状。     

合并脊髓静脉高压综合征的脊髓血管母细胞瘤的手术治疗

目的 总结合并脊髓静脉高压综合征（VHM）的脊髓血管母细胞瘤的诊治经验。方法 回顾性分析2005~2019年手术治疗的10例脊髓血管母细胞瘤的临床资料,结合术前MRI T2像及病人临床表现评估VHM,并总结脊髓血管母细胞瘤合并VHM的诊治经验。结果 单发肿瘤9例,其中肿瘤位于颈段3例、胸段4例、腰段2例;多发1例（颈段、胸段及腰段均有）。术前存在VHM 6例,术前MRI均可见病变节段以外的脊髓中央区MRI T2高信号,存在明显的脊髓功能损伤症状,如肢体运动功能障碍或大小便功能障碍等。10例肿瘤均全切除,其中1例多发肿瘤一次切除3个胸段肿瘤。无手术死亡病例。结论 脊髓血管母细胞瘤为富含血管的血管性肿瘤,往往有高流量的血流通过,引流静脉常迂曲扩张并同脊髓表面正常引流静脉沟通导致VHM,有症状者应早期手术,避免出现不可逆的神经功能损伤,肿瘤切除后VHM自然治愈。     

中枢神经系统血管母细胞瘤的显微外科治疗

目的探讨中枢神经系统血管母细胞瘤的临床特征、诊断和鉴别诊断．以及外科手术治疗特点。方法对1999年2月-2006年5月施行手术治疗的21例中枢神经系统血管母细胞瘤患者（肿瘤位于小脑半球者14例，第四脑室内延髓背侧2例，延髓下部1例和脊髓内4例）的临床资料进行回顾性总结与分析。结果手术全切除肿瘤患者14例，次全切除7例，其中2例于手术前施行微导管超选择性肿瘤供血动脉栓塞术。手术后神经系统症状明显改善18例，2例因脑积水症状未改善行侧脑室．腹腔分流术，1例手术后并发出血，再次手术。12例患者平均随访25个月，9例恢复正常工作和生活，1例遗留小脑性共济失调，2例肿瘤复发（1例再次手术全切除肿瘤；1例多部位复发而行γ-刀治疗。随访7个月肿瘤无增大）。本组无死亡病例。结论 中枢神经系统血管母细胞瘤为良性肿瘤，肿瘤全切除可获得根治。应用显微外科技术、手术前供血动脉栓塞以及立体定向放射外科等综合措施可提高疗效。     

脊髓髓内血管母细胞瘤的显微外科治疗

目的总结脊髓髓内血管母细胞瘤的显微外科手术治疗经验。方法回顾性分析2005年1月至2013年11月显微手术治疗的16例脊髓髓内血管母细胞瘤患者的临床资料。结果肿瘤全切除15例（18个肿瘤,2例多发）,部分切除1例;术后病理学检查证实为血管母细胞瘤。术后11例随访3月∽5年,无复发,病情恢复良好。结论对于脊髓髓内血管母细胞瘤,显微镜下严格沿正确的界面进行分离,先离断动脉后处理静脉,避免分块切除而力争全切,是减轻术中出血和神经功能损害的关键。虽然手术风险高,但对临床表现明显的患者,宜应行积极的显微外科手术治疗,防止症状发展。     

脊髓髓内动静脉畸形的复合手术治疗

目的 探讨脊髓髓内动静脉畸形（IAVMs）的临床特点以及复合手术的临床疗效。方法 回顾性分析2017年9月至2021年6月在复合手术室行显微手术治疗的7例IAVMs的临床资料。先行DSA定位，显微镜下完全显露畸形血管团，术中DSA美兰实时显影技术精准判断供血动脉、引流静脉。结果 7例均顺利完成手术，其中6例畸形血管团全切除，术后即刻DSA显示供血动脉、畸形血管团、引流静脉不显影；1例胸段IAVM因部分病灶深入脊髓腹侧，在阻断供血动脉及引流静脉后仅进行部分切除。7例术后随访9~54个月，平均（27.7±16.5）个月；术后9个月McCormick分级Ⅰ级2例，Ⅱ级2例，Ⅲ级1例，Ⅳ级2例；末次随访，7例症状均改善，无死亡病例；除部分切除的1例外，其余6例复查MRI未见迂曲血管影及畸形血管团。结论 IAVMs临床少见。在电生理监测下，运用术中DSA美兰实时显影辅助显微手术切除IAVMs，可取得较好的临床疗效。     

脊髓血管母细胞瘤的显微手术治疗

目的总结脊髓血管母细胞瘤的治疗经验及疗效。方法回顾性分析38例脊髓血管母细胞瘤病人的手术经验。均在显微镜下行肿瘤切除,术前及术后1周采用McCormick分级进行脊髓功能评估。结果肿瘤全切除37例,近全切除1例。术后运动及感觉障碍改善30例,无变化6例,加重2例(1个月内恢复)。无手术死亡病例。随访3～60个月,脊髓功能改善2例,无明显变化36例;未见肿瘤复发。结论血管母细胞瘤为高度血管化的良性髓内肿瘤,可通过显微镜下全切除治愈。     

脊髓血管母细胞瘤的诊断和显微手术治疗

目的探讨脊髓血管母细胞瘤的诊断和显微手术治疗技巧.方法回顾性分析11例脊髓血管母细胞瘤病人的临床特征、影像学诊断、治疗方法和预后,均行显微外科手术切除肿瘤.结果肿瘤均全切除.病人平均住院15d,术后短时间内症状改善8例,无变化3例;无手术死亡病例.8例随访6～36个月,无肿瘤复发,神经功能均获得不同程度改善.结论MRI对脊髓血管母细胞瘤的诊断是必要的.熟练的显微外科手术技术和丰富的髓内肿瘤切除经验对手术成功非常关键.     

Spinal Cord Hemangioblastoma : Diagnosis and Clinical Outcome after Surgical Treatment

Objective

Spinal cord hemangioblastoma is an uncommon vascular neoplasm with a benign nature and is associated with von Hippel-Lindau (VHL) disease in 20-30% of patients. Total removal of these tumors without significant neurological deficit remains a great challenge. The purpose of this study was to investigate the efficacy of VHL mutation analysis and to evaluate surgical outcome of patients with spinal cord hemangioblastomas.

Methods

This study included nine patients treated for spinal cord hemangioblastomas at our institute between December 1994 and March 2006. There were four male and five female patients. Mean age was 37.8 years. The mean follow-up period was 22.4 months. Magnetic resonance imaging (MRI) of the complete neuraxis was done in all cases and VHL mutation analysis was performed in three cases for a definite diagnosis.

Results

Six patients had intramedullary tumor, and the remaining patients had intradural extramedullary lesions. Five patients were associated with VHL disease. The von Hippel-Lindau mutation analysis was done in three patients and two of them showed VHL gene abnormality. Tumors were located in the cervical cord in five cases and in the thoracic cord in four cases. All patients underwent surgical intervention, and total removal was achieved in six cases. All patients showed improvement or, at least, clinically stationary state. Surgical complications did not develop in any cases.

Conclusion

Spinal hemangioblastoma in this series has been safely and effectively removed via a posterior approach. Postoperatively, clinical outcome was excellent in the majority of cases. The VHL mutation analysis was useful in patients with family history and in those with multiple hemangioblastomas.     

脑血管母细胞瘤的外科治疗(附38例报告)

目的总结、探讨脑血管母细胞瘤的外科治疗。方法对38例脑血管母细胞瘤的临床表现、手术治疗进行回顾分析。结果30例囊性肿瘤均行瘤结节全切除术;随防3～20年,未复发29例。8例实体性肿瘤中,4例先行血管内栓塞后行全切除术,另4例因术中大出血行大部分和部分切除;随访3～12年,全切除的4例未复发,非全切除者1例无变化,1例增大,2例死亡。结论囊性血管母细胞瘤外科治疗易做到全切除,疗效好。实体性血管网状细胞瘤外科治疗因血管丰富,可先行血管内栓塞,减少术中出血量,提高切除率。     

Surgical treatment of hemangioblastomas of the central nervous system in pediatric patients

Objective Hemangioblastomas are histologically benign lesions that occur sporadically or as a manifestation of von Hippel–Lindau disease (VHL). The treatment strategy of these neoplasms is complicated by their unpredictable growth patterns and the often irreversible neurological deficits they may cause. This study aims to outline the neurosurgical treatment options and to address the ongoing debate of surgical timing in pediatric patients with VHL.Patients and methods Thirteen consecutive pediatric patients (mean age 15.1 years) who were surgically treated for intracranial (n=8) or spinal hemangioblastomas (n=5) were included in this study (range of clinical follow up 12–86 months). Ten patients were affected by von Hippel–Lindau and three were with sporadic tumors. Serial examinations, preoperative MRI studies, and operative findings were reviewed.Results Patients with cerebellar and intramedullary hemangioblastomas did not develop additional neurological deficits postoperatively. Two patients with brainstem tumors exhibited transient hemiparesis and caudal nerve palsy, respectively. Both patients recovered completely from their postoperative deficits. Preoperatively symptomatic patients with spinal tumors did not deteriorate nor improve after surgery. During the observed follow-up periods, no tumor recurrences were observed.Conclusion Central nervous system (CNS) hemangioblastomas in pediatric patients can be surgically treated with low morbidity. Based on our experience, we recommend considering also the surgical removal of asymptomatic hemangioblastomas with proven radiological progression to prevent the development of permanent neurological deficits. Molecular screening of every pediatric patient and family is mandatory to enable the detection of extraneurological tumors and the development of an efficient therapeutic strategy.     

散发性与VHL相关性颅内血管母细胞瘤的MRI与病理相关性研究

目的 探讨散发性与Von Hippel-Lindau(VHL)病相关性颅内血管母细胞瘤的MRI表现类型及其病理基础.方法 2002年至2009年经手术病理证实的颅内血管母细胞瘤患者29例,将肿瘤MRI影像表现特点与手术病理结果做对照分析.结果 29例颅内血管母细胞瘤患者,散发性17例(59%),VHL病12例(41%),共计66个血管母细胞瘤,MRI表现为实性小结节型(28个)、实性肿块型(18个)及囊结节型(20个),病理光镜下表现为网状型、细胞型及混合型.结论 血管母细胞瘤的MRI表现类型与其病理基础密切相关.对VHL病患者颅内无症状的实性小结节型血管母细胞瘤应长期追踪观察.     

Diagnosis and microsurgical treatment of spinal hemangioblastoma

Spinal cord hemangioblastomas are rare benign tumors, with difficult surgical management and poor prognosis due to high vascularization. We aim to evaluate the diagnostic methods and microsurgical treatment of spinal cord hemangioblastoma. This retrospective study assessed 25 patients treated for spinal hemangioblastoma using microsurgery at Beijing Tiantan Hospital and Department of Neurosurgery, The General Hospital of Chinese People’s Armed Police Forces, between October 2008 and October 2013. Clinical, imaging, and treatment data were collected. Meanwhile, efficacy was assessed with the McCormick grading system for spinal cord function. The symptoms lasted 17.0 ± 15.1 months. Sixteen (64 %) patients were suffering from von Hippel-Lindau disease; magnetic resonance imaging revealed the lesions in all patients. Intraoperative fluorescence angiography was helpful in identifying the feeding arteries and draining veins. Total tumor removal was achieved in all subjects. Patients were followed up for 21.3 ± 8.5 months. One week after surgery, neurological symptoms were improved in 22 patients, remained stable in 2 patients, and were aggravated in 1. The latter patient began to recover 7–10 days after surgery and was completely recovered within a month. At the last follow-up, all patients were alive, and all showed a McCormick grade ≤II. Microsurgery seems effective in the treatment of spinal cord hemangioblastoma. Intraoperative fluorescence angiography is helpful in defining the resection scope, to reduce intraoperative bleeding and prevent spinal swelling, which results in improved success rate.     

Management of von Hippel-Lindau disease-associated CNS lesions

Patients with von Hippel-Lindau disease (VHL) often harbor significant disease burden within the CNS, specifically craniospinal-axis hemangioblastomas and endolymphatic sac tumors (ELSTs). The majority (60-80%) of patients with VHL harbor hemangioblastomas, and 10-15% will develop ELSTs. Advances in the understanding of the natural history and outcomes associated with the surgical management of VHL-associated tumors have led to improved management of patients with VHL. Optimizing indications for surgical intervention and refining of surgical techniques for these lesions can reduce patient morbidity associated with the management of this syndrome. In this article, we review the various aspects of perioperative management of patients with VHL, surgical indications and general operative principles for the management of hemangioblastomas and ELSTs, and outcomes associated with the surgical treatment of these tumors.     

脊髓型囊虫病3例报告并文献回顾

目的回顾分析脊髓型囊虫病的临床表现、诊断和治疗,提升对该病的总体认识及诊疗水平。方法回顾3例脊髓型囊虫病病例的临床资料并复习相关文献,患者均行后正中入路椎管内肿物全切除术。结果 3例均位于胸段脊髓,病理证实为脊髓囊虫,术后2例浅感觉减退,治疗后恢复。术后肌力较术前恢复,继续口服驱虫药物。结论脊髓型囊虫病发生率低,手术切除是首选治疗方法,术后建议用药物抗囊虫治疗。     

Operative management of brainstem hemangioblastomas

Brainstem hemangioblastomas are technically challenging lesions. The authors retrospectively analyzed their experience in 24 patients with brainstem hemangioblastomas to evaluate the management strategies used over time and the results of microsurgical treatment. All patients were operated on between 2007 and 2012. The patients received postoperative follow-up by neuroradiological and neurological examinations. The maximum diameter of the tumors ranged from 2.0 to 4.5 cm (mean 3.6 cm). Gross total resection was achieved in 24 patients (100%). Two patients (8%) had new neurological deficits or worsening of pre-existing deficits. One patient (4%) died because of brain stem dysfunction after the operation. Radical en bloc surgical resection of brainstem hemangioblastomas in symptomatic patients is a safe and effective primary treatment. Preoperative embolization is not necessary. It is very important and necessary to differentiate and dissect precisely at the interface of the tumor surface and the brainstem with a meticulous microsurgical technique until the tumor is removed en bloc. The preoperative neurological status of the patient predicts the postoperative functional outcome. Asymptomatic patients with hemangioblastoma may be followed clinically with MRI surveillance at regular intervals.     

中枢神经系统血管母细胞瘤312例临床分析及长期随访

目的　探讨医学发展对本病诊治的影响。方法　回顾性总结分析上海华山神经外科集团医院 1974年 5月至 2003年 12月收治的 312例中枢神经系统血管母细胞瘤病人的临床资料,分3个时期比较其诊断与治疗进展情况,说明近年来医学发展对本病诊治的影响。结果　本组 312例中,男 188例,女 124例,男 女=1 52 1;年龄 14~72岁,平均 39岁;多发性肿瘤 20例,共 331个肿瘤,最常见于小脑半球或蚓部,其次是脊髓,亦可偶见于幕上;有明确家族史者 19例 ( 6. 1% ),确诊VHL病 20例(6. 4% )。在前期(1974年 5月至 1989年 7月 )CT和气脑、脑室造影是主要诊断方法,术前确诊率 42%;中期(1989年 8月至 1996年 12月 )和后期 ( 1997年 1月至 2003年 12月 )CT和MRI是主要诊断手段,术前确诊率分别为 58%、92%;后期还开展术前栓塞治疗。肿瘤全切率三个时期分别为 83%、80. 5%、97 .9%,术后死亡率分别为 8%、7. 8%、3 .1%。长期随访KPS>80分者三组分别为 88. 5%、92.1%、79 .7%,死亡率分别为 8 .2%、0、3. 1%。结论　本病诊断主要依靠MRI,对可疑病人应做DSA检查,但早期诊断仍存在问题;手术治疗可靠有效,肿瘤全部切除可治愈本病;但实质性、家族性、多发性中枢神经系统血管母细胞瘤 (HB)特别是位于脑干、脊髓时治疗仍较困难;家族性HB(VHL病)     

硬脊膜外囊肿

硬脊膜外囊肿罕见。本文报告了过去３５年间沈阳军区总医院收治的６例病人，并结合文献对其发病机制、病理、临床特征、放射学影象、ＭＲＩ影象及手术要点等加以讨论。认为本病的ＭＲＩ影象具有特征性，它有助于本病的术前诊断，使手术效果改善。