岩骨尖上斜坡区良性肿瘤的显微外科治疗

目的：介绍２１例岩骨尖上斜坡区良性肿瘤的显微外科治疗结果，就其手术入路进行讨论。方法：分析肿瘤的临床特点、神经放射学资料，选择相应的手术入路。结果：肿瘤经颞下天幕上入路切除８例，天幕上下联合入路切除７例，枕下入路切除６例。肿瘤全切率７６．２％。术后死亡率４．８％。结论：根据肿瘤的性质、大小和部位，选择相应的手术入路是十分重要的。联合入路在巨大肿瘤的切除中最为常用     

岩斜区肿瘤的手术入路选择

目的探讨岩斜区肿瘤的手术入路选择,以提高岩斜区肿瘤的手术疗效。方法回顾性分析2000年1月至2009年12月经显微外科技术切除的92例岩斜区肿瘤,比较手术入路对手术结果的影响。根据肿瘤的临床和影像学特征,将岩斜区肿瘤分为四型。Ⅰ型,采用颞下-经天幕入路;Ⅱ型,采用颞下-经岩骨嵴入路,另有3例巨大型蝶岩斜坡型脑膜瘤采用经岩入路(幕上幕下联合或乙状窦前入路);Ⅲa,采用枕下乙状窦后入路;Ⅲb,采用乙状窦后-内听道上入路;Ⅳ型,经鼻-蝶入路切除。结果肿瘤SimpsonⅠ～Ⅱ级全切除83例。次全切除9例,其中Ⅰ型1例,Ⅱ型5例,Ⅲb型1例,Ⅳ型2例。术后新增脑神经功能障碍16例(17.4%),肢体偏瘫2例;另有2例KPS评分为50分,这2例随访3个月后基本恢复至术前状态。无死亡病例。结论对于不同类型的岩斜区肿瘤,选择合适的手术入路有助于提高疗效,减少术后并发症。乙状窦后及其改良入路、颞下-经天幕及其改良入路是岩斜区重要的手术入路。而硬膜外岩斜区肿瘤适合于采用经蝶入路手术切除。     

33例岩斜区肿瘤的显微外科治疗

目的探讨岩斜区肿瘤的显微切除技术。方法我科1997年8月至2006年12月采用显微外科技术切除33例岩斜区肿瘤，主要采用岩骨乙状窦前入关路、颞下经小脑幕入路、乙状窦后入路、幕上下联合入路4种入路。结果肿瘤全切除22例（66.7％），次全切7例，大部分切除4例。术后症状和体征完全消失13例，症状较术前减轻12例，颅神经症状同术前2例，出现新的神经功能障碍6例。结论术前充分的准备、选择合适的手术入路和娴熟的显微外科技术可以减少手术并发症的发生，降低病残率。     

经岩骨乙状窦前幕上下联合入路切除岩斜区肿瘤

目的通过对31例岩斜区肿瘤显微外科治疗的分析，探讨幕上下经岩骨乙状窦前入路切除岩斜区肿瘤的优越性。方法分析1998年12月至2005年10月经不同手术入路显微外科切除的31例岩斜区肿瘤．总结其临床表现、神经影像学特征及握微手术方法、手术效果和术后处理。结果31例病人经乙状窦前入路手术15例，其中肿瘤全切除8例，全切率为533％，出现并发症9例（60．0％），死亡2例（133％）。经其它入路手术16例，其中肿瘤全切2例（12．5％），出现并发症10例（62．5％），死亡3例（18．8％）。与其他入路手术相比，经乙状窦前入路可明显提高肿瘤全切率（P〈0.05），而手术并发症率及死亡率无屁著性差别。结论与传统手术入路相比，经岩骨乙状窦前入路可充分显露岩斜区病变，手术全切除率明屁高于其它入路。     

显微手术切除岩斜区巨大脑膜瘤的策略和技巧

目的 探讨显微手术切除岩斜区巨大脑膜瘤的策略和技巧。方法 总结35例岩骨头斜坡区巨大脑膜瘤的临床表现，显微手术方法、手术结果和术后处理。结果 肿瘤全切除21例（60%），死亡2例（5.7%），其中经岩骨乙状窦前幕上下联合入路22例，全切除18例（82%），无死亡。结论 根据术前与术中的策略，选择好手术入路和运用好显微外科术可以提高岩骨尖斜坡巨大脑膜瘤的切除率，降低手术病死率。     

岩斜区脑膜瘤的显微外科治疗

目的通过对234例岩斜区脑膜瘤显微外科治疗的分析,探讨岩斜区脑膜瘤的显微手术入路与治疗效果。方法总结234例岩斜区脑膜瘤的临床表现、神经影像学特征、显微手术入路及方法和术后处理。结果肿瘤全切除及次全切除192例(82.0%),死亡4例(1.7%)。结论岩斜区脑膜瘤的治疗采用手术治疗,根据肿瘤在斜坡的不同部位采用相应的手术入路,经岩骨乙状窦前入路目前是该部位肿瘤的首选手术入路,早期诊断、早期治疗,减少手术并发症,以达到治愈的目的。     

基于影像学分型的个体化手术治疗岩斜区脑膜瘤

目的 探讨依据岩斜区脑膜瘤的影像学分型进行个体化手术治疗的效果。方法 回顾性分析2010年1月~2017年12月收治的107例岩斜区脑膜瘤的临床资料。依据术前影像学分型选择手术入路:岩尖型16例中,11例颞下经天幕入路,5例岩前即Kawase入路;海绵窦型19例中,13例Kawase入路,5例乙状窦后入路,1例因累及颞下窝行Fisch颞下窝A型入路;天幕型38例中,6例颞下经天幕入路,13例乙状窦前入路,19例乙状窦后入路;上斜坡型34例中,21例乙状窦前入路,7例乙状窦后入路,4例Kawase入路,2例颞下联合乙状窦后入路。结果 16例（100%）岩尖型、38例（100%）天幕型、32例（94.1%）上斜坡型及10例（52.6%）海绵窦型达到全切除或次全切除,仅9例（47.4%）海绵窦型和2例（5.9%）上斜坡型行大部分切除。术后新发神经功能障碍27例（25.2%）,无手术死亡病例。术后6个月KPS评分[（77.6±11.8）分]与术前[（74.3±15.0）分]无统计学差异（P>0.05）。结论 对于岩斜区脑膜瘤,依据术前影像学分型采取个体化手术入路,结合熟练的颅底解剖及娴熟的显微手术技巧,可达到最大程度切除肿瘤和尽可能减少术后神经功能障碍之间的平衡。     

颞枕经小脑幕入路显微手术切除岩斜区脑膜瘤

目的 探讨颞枕经小脑幕人路切除岩斜区脑膜瘤的可行性.方法 对18例经颞枕入路切除的岩斜区脑膜瘤患者进行回顾性研究.全部病例均在术前行MRI检查.结果 18例于术患者,肿瘤直径<3.0cm4例;3.0-4.5cm5例;>4.5 cm 9例,最大径可达到7.6 cm×7 cm X7 cm.全切8例,次全切9例,大部分切除1例.偏瘫2例,面瘫1例.听力减退1例,无脑脊液耳漏、失语.1例死亡.结论 颞枕经小腩幕入路切除岩斜区脑膜瘤具有可行性,可以使鞍旁海绵窦区、上中斜坡、岩骨背侧小脑脑桥角区暴露充分,利十该区域占位性病变的手术治疗.     

手术治疗岩斜区脑膜瘤

目的 提高岩斜区脑膜瘤的切除程度与术后疗效。方法 回顾性分析22例岩斜区脑膜瘤的临床资料。结果 全组病例获肿瘤全切除者12例，肿瘤次全切除6例，肿瘤大部分切除4例；其中经乙状窦前入路手术治疗的11例中，获全肿瘤切除者8例，次全肿瘤切除2例，肿瘤大部切除1例。结论 岩斜区脑膜瘤宜行积极手术治疗；乙状窦前入路是目前手术治疗岩斜区脑膜瘤的最佳入路；掌握手术方法和术中注意事项，有利于提高肿瘤切除程度和术后疗效；肿瘤大小、质地及其与邻近结构的相互关系，也是影响岩斜区脑膜瘤患者术后疗效的重要因素。     

显微手术治疗岩斜区脑膜瘤

目的通过对28例岩斜区脑膜瘤的显微手术治疗，探讨岩斜区脑膜瘤经岩骨前入路和岩骨后入路手术治疗的效果。方法28例均采用显微手术治疗，采用传统的经乙状窦前入路9例，颞下经小脑幕入路4例，经乙状窦后入路9例，经改良的乙状窦前入路6例。结果肿瘤全切除16例，次全切除9例，大部切除3例，无死亡病例。结论显微手术治疗岩斜区脑膜瘤，根据肿瘤在岩斜区的不同位置采用相应的手术入路，以减少手术并发症，达到治愈的目的。     

SLC44A1–PRKCA fusion in papillary and rosette-forming glioneuronal tumors

We investigated the fused protein of solute carrier family 44 choline transporter member 1 (SLC44A1) and protein kinase C alpha (PRKCA) in three patients with papillary glioneuronal tumors (PGNT). PGNT and rosette-forming glioneuronal tumors (RGNT) are recently identified, unusual glioneuronal tumor variants which were categorized as novel tumor entities in the 2007 World Health Organization classification system. The molecular background of these tumors remains poorly understood due to the paucity of studies. The SLC44A1–PRKCA fusion was recently detected in three cases of PGNT. We invesitgated for the SLC44A1–PRKCA fusion protein in the three PGNT patients and a further two with RGNT using fluorescence in situ hybridization. Two out of the three PGNT patients had a fused signal (paired red–green signal) representing a rearrangement on chromosomes 9 and 17. A normal signal pattern was observed in the third PGNT patient. Neither of the two RGNT patients demonstrated a fused signal. This suggests that the SLC44A1–PRKCA fusion is a characteristic alteration in PGNT but not RGNT. Therefore, it is a potential biomarker of PGNT. The paired red–green signal that was observed in the PGNT patients implies the presence of a different breakpoint than that previously reported in the 9q31 and 17q24 genes.     

Malignant triton tumor in the thoracic spine

We present a 15-year-old patient diagnosed with peripheral neurofibromatosis (NF-1), who was admitted with paraparesis caused by a large intrathoracic tumor with an intracanalicular component that affected the spinal cord. After surgery his condition improved, but a year later he suffered a relapse and died. Histologically the tumor was diagnosed as malignant with neurogenic and myogenic differentiation (”malignant triton tumor”). Malignant triton tumors (MTT) are infrequent; those found in the head and neck and the upper or lower extremities have a better prognosis than those in the retroperitoneum, buttock, or trunk. It is not clear whether this variation is due to a difference in tumor grade, stage, or resectability, or whether it is a consequence of therapy. Received: 23 August 1998 Revised: 3 September 1999     

Leptomeningeal disease in chronic lymphocytic leukemia

Chronic lymphocytic leukemia (CLL) is the most common lymphoproliferative disorder in the western hemisphere, with an annual incidence of 3:100 000. Commonly patients are asymptomatic but not rarely disease progression occurs in the setting of lymphadenopathy and extensive leukemic burden. Leptomeningeal involvement in patients with CLL is infrequent, with presenting symptoms of headache (23%), acute or chronic changes in mental status (28%), cranial nerve abnormalities (54%) including optic neuropathy (28%), weakness of lower extremities (23%) and cerebellar signs (18%). In this report, we discuss a CLL patient with leptomeningeal involvement, who presented with neurological symptoms as the first clinical sign, and a diagnosis of leptomeningeal was made based on CSF cytology and flow cytometry. Treatment consisted of radiation therapy and intrathecal chemotherapy with arabinoside–cytosine and systemic chemotherapy. On the basis of this patient-report together with 37 other previously reported cases, the clinical characteristics together with treatment options and outcome of leptomeningeal involvement in CLL are reviewed. Our case together with data from the literature indicate that a timely diagnosis and intensive treatment of leptomeningeal disease of CLL may lead to longstanding and complete resolution of neurological symptoms.     

脑瘤病人胎免疫组织细胞移植与肿瘤相关抗原

目的:探讨肿瘤相关抗原在脑瘤病人胎免疫组织细胞移植后变化特点及疗效评估。方法:采用放射免疫测定法对42例接受胎胸腺组织、肝脾细胞移植的脑瘤病人作血清甲胎蛋白(AFP)、癌胚抗原(CEA)和铁蛋白(FT)等肿瘤相关抗原测定。采用荧光抗体技术检测抗胎胸腺抗体,严密观察病人的临床症状及体征。将亚恶性脑瘤(包括良性脑瘤)与恶性脑瘤相比较,并将病人移植前、移植后的测定值作自身对照统计学处理。结果:AFP未呈现显著性差异(P>0.05);而CEA和FT则呈现极显著性差异(P<0.01),肿瘤相关抗原水平的测定,反应了不同脑瘤的特点,有助于脑瘤性质的鉴别和预后的评估。移植后病人经抗胎胸腺抗体检测,结果阴性;临床观察也未见任何免疫排斥反应或移植物抗宿主反应(?)象。结论:本组病人移植后临床症状不断改善,肿瘤相关抗原明显降低,表明移植治疗效果是满意的。     

磁共振成像预测颅内肿瘤质地的研究进展

术前对颅内肿瘤质地的准确评估对于手术入路的选择以及预后的评估极为重要。目前,对颅内肿瘤质地的预测方法主要是通过磁共振成像。本文分别对磁共振常规序列、弥散序列、弹性序列及其他序列预测肿瘤质地研究相关的文献进行综述和分析。我们认为常规序列和弥散序列预测肿瘤质地的有效性尚存在争议;磁共振波谱、灌注加权成像及稳态进动平衡序列目前研究数据较少,不能得出有效的结论;弹性序列是目前预测肿瘤质地最有效的评价指标。     

经胸联合后正中入路切除胸椎哑铃型神经源性肿瘤

目的 探讨胸椎哑铃型神经源性肿瘤的手术方法。方法 1991年3月至1999年3月经胸联合后正中入路一期切除胸椎哑神经源性肿瘤7例,其中神经纤维瘤4例,神经鞘瘤2例,恶性神经鞘瘤1例。结果 6例获肉眼下全切除,1例次全切除,所有患症状均不同程度改善。随访6例,平均3.5年,5例正常生活、工作,1例恶性神经鞘瘤3例前次全切除刚结束放射治疗,未见复发。结论 经胸联合后正中入路能满意暴露肿瘤,安全切除复杂的胸椎哑铃型神经源性肿瘤。     

Brachial Plexus Tumors in a Consecutive Series of Twenty One Patients

Objective

This is a retrospective review of 22 surgically treated benign and malignant tumors of brachial plexus region to describe clinical presentation, the characteristics of brachial plexus tumor and clinical outcomes with a literature review.

Methods

Twenty-one patients with consecutive 22 surgeries for primary brachial plexus tumors were enrolled between February 2002 and November 2011 were included in this study. The medical records of all patients were reviewed.

Results

Eleven male and 10 female patients were enrolled. Mean age was 39 years. Three patients had brachial plexus tumor associated with neurofibromatosis (13.6%). Presenting signs and symptoms included parenthesis and numbness (54.5%), radiating pain (22.7%), direct tenderness and pain (27.2%), palpable mass (77.3%). Twelve patients presented preoperative sensory deficit (54.5%) and 9 patients presented preoperative motor deficit (40.9%). Twenty tumors (90.9%) were benign and 2 tumors (9.1%) were malignant. Benign tumors included 15 schwannomas (68.2%), 4 neurofibromas (18.2%) and 1 granular cell tumor (4.5%). There were 1 malignant peripheral nerve sheath tumor (MPNST) and 1 malignant granular cell tumor. Gross total resection was achieved in 16 patients (72.7%), including all schwannomas, 1 neurofibroma. Subtotal resection was performed in 6 tumors (27.3%), including 3 neurofibromatosis associated with brachial plexus neurofibromas, 1 MPNST and 2 granular cell tumor in one patient.

Conclusion

Resection of tumor is the choice of tumor in the most of benign and malignant brachial plexus tumors. Postoperative outcomes are related to grade of resection at surgery and pathological features of tumor.     

Response to combined BRAF/MEK inhibition in adult BRAF V600E mutant spinal pilocytic astrocytoma

Pilocytic astrocytomas are World Health Organisation (WHO) grade I tumors, occurring predominantly supratentorially and in the pediatric population. Although the mainstay of treatment is local therapies such as surgery, targeted systemic therapies may be necessary for recurrent or unresectable disease. The majority of sporadic pilocytic astrocytomas are associated with the BRAF-KIAA fusion gene, which results in constitutive activation of the MAP Kinase pathway. Less frequently, the BRAF V600E point mutation has been described, occurring in less than 10% of supratentorial pilocytic astrocytomas. Tumours with this mutation may respond to targeted therapy against the BRAF/MAP Kinase pathway. We report the first described case of a spinal pilocytic astrocytoma in an adult patient with a BRAF V600E mutation responding to targeted therapy using BRAF and MEK tyrosine kinase inhibitors, and share our experiences with the management of toxicity in this patient population.     

Radical surgery of thalamic tumors in children

The authors review eight cases of thalamic tumors in children. Radical surgery was performed in seven cases. All cases were diagnosed bycomputed tomographic scanning, and histological diagnosis showed seven benign astrocytomas and one malignant astrocytoma. All patients are still alive. The treatment of these tumors is discussed.