PURPOSE:Angiocentric glioma (AG), a benign tumor identified within the last two decades, was officially included in the 2007 WHO Classification of Tumors of the Central Nervous System, WHO grade I. The tumor is relatively rare, with only approximately 100 cases reported. We aim to complement the characteristics and long-term prognosis of AG, as well as to detect MYB-QKI fusions. METHODS:The characteristics of all cases collected between 1 March 2009 and 1 March 2023 at the Beijing Sanbo Brain Hospital, Capital Medical University, were summarized and analyzed. Additionally, all fourteen patients were tested for MYB-QKI fusions. RESULTS:AG more predominantly occurs in adolescents (median age 16.5-year-old), and commonly presents with drug-resistant epilepsy. AG is frequently localized in the supratentorial regions and only one patient is in the brainstem. Brain parenchyma atrophy, and stalk-like signs can observe in imaging. Pathologically, tumor cells are perivascular pseudorosettes, presenting immunoreactivity for GFAP, S-100, Vimentin, "dot-like" staining for EMA, and low proliferative activity. Focal cortex dysplasia was observed in four patients. Twelve of fourteen (85.7%) patients were found with MYB-QKI fusions. Completely surgical resection typically has a satisfactory prognosis with long-term follow-up. CONCLUSION:AG is a rare benign tumor with a favorable prognosis after complete resection, characterized by refractory epilepsy, frequently occurring in adolescents. MYB-QKI fusions were detected in most AG patients, as a good defining genetic alteration pathologically. The potential presence of focal cortical dysplasia (FCD) may affect the prognosis of epilepsy.

Current histological classification of low-grade glioneuronal tumours does not adequately represent their underlying biology. The neural lineage(s) and differentiation stage(s) involved and the cell state(s) affected by the recurrent genomic alterations are unclear. Here, we describe dysregulated oligodendrocyte lineage developmental programmes in three low-grade glioneuronal tumour subtypes. Ten dysembryoplastic neuroepithelial tumours, four myxoid glioneuronal tumours and five rosette-forming glioneuronal tumours were collected. Besides a comprehensive characterization of clinical features, known diagnostic markers and genomic alterations, we used comprehensive immunohistochemical stainings to characterize activation of rat sarcoma/mitogen-activated protein kinase pathway, involvement of neuronal component, resemblance to glial lineages and differentiation blockage along the stages of oligodendrocyte lineage. The findings were further complemented by gene set enrichment analysis with transcriptome data of dysembryoplastic neuroepithelial tumours from the literature. Dysembryoplastic neuroepithelial tumours, myxoid glioneuronal tumours and rosette-forming glioneuronal tumours occur at different ages, with symptoms closely related to tumour location. Dysembryoplastic neuroepithelial tumours and myxoid glioneuronal tumours contain oligodendrocyte-like cells and neuronal component. Rosette-forming glioneuronal tumours contained regions of rosette-forming neurocytic and astrocytic features. Scattered neurons, identified by neuronal nuclei antigen and microtubule-associated protein-2 staining, were consistently observed in all dysembryoplastic neuroepithelial tumours and myxoid glioneuronal tumours examined, but only in one rosette-forming glioneuronal tumour. Pervasive neurofilament-positive axons were observed only in dysembryoplastic neuroepithelial tumour and myxoid glioneuronal tumour samples. Alterations in B-Raf proto-oncogene, serine/threonine kinase, fibroblast growth factor receptor 1, fibroblast growth factor receptor 3 and platelet-derived growth factor receptor alpha occurred in a mutually exclusive manner, coinciding with strong staining of phospho-p44/42 mitogen-activated protein kinase and low apoptotic signal. All dysembryoplastic neuroepithelial tumours, myxoid glioneuronal tumours and the neurocytic regions of rosette-forming glioneuronal tumours showed strong expression of neuron-glia antigen 2, platelet-derived growth factor receptor alpha (markers of oligodendrocyte precursor cells) and neurite outgrowth inhibitor-A (a marker of developing oligodendrocytes), but lacked the expression of oligodendrocyte markers ectonucleotide pyrophosphatase/phosphodiesterase family member 6 and myelin basic protein. Notably, transcriptomes of dysembryoplastic neuroepithelial tumours were enriched in oligodendrocyte precursor cell signature, but not in signatures of neural stem cells, myelinating oligodendrocytes and astrocytes. Dysembryoplastic neuroepithelial tumour, myxoid glioneuronal tumour and rosette-forming glioneuronal tumour resemble oligodendrocyte precursor cells, and their enrichment of oligodendrocyte precursor cell phenotypes is closely associated with the recurrent mutations in rat sarcoma/mitogen-activated protein kinase pathway.

Craniopharyngioma (CP) is a mostly benign tumor that is nonetheless one of most formidable skull base lesions. CP tends to recur, and scarce clinical results are available regarding its long-term outcomes. From February 1996 to April 2002, craniopharyngiomas primarily resected by open surgery in a single-center and single-surgeon practice were screened. Medical information regarding patients’ baseline, tumor parameters, endocrinological results, complications, and quality of life in those patients with a follow-up longer than 20 years were reviewed. Nineteen out of 187 patients who met the inclusion criteria were eligible, and 78.9

Objective:To investigate the surgical method and curative effect of resection of craniopharyngioma through sub-frontal longitudinal fissure duramater-keyhole approach.Methods:The clinical data, imaging data of 179 patients with craniopharyngioma treated by sub-frontal longitudinal fissure duramater-keyhole approach from January 2017 to January 2021 in Sanbo Brain Hospital Capital Medical University were studied retrospectively.Results:Through this approach, total resection in 175 cases (97.77%), near total resection in 2 cases (1.12%), partial resection in 2 cases (1.12%); drilling of the tuberculum sellae in 31 cases (17.32%); the pituitary stalk was reserved in166 cases (92.74%), preserved in 13cases (7.26%); papillary craniopharyngioma in 27 cases (15.08%), ameloblastic craniopharyngioma in 152cases (84.92%); postoperative visual acuity improved in 65 cases (36.31%), not improved in 53 cases (29.61%), decreased in 6 cases (3.35%); electrolyte disorder in 101 cases (56.42%), polydipsia and polyuria in 65cases (36.31%), cerebrospinal fluid rhinorrhea in 3 cases (1.68%), hydrocephalus in 6 cases (3.35%). Postoperative hematoma in 2 (1.12%) cases and cerebral infarction in 6 cases (3.35%), intracranial infection in 8 cases (4.47%), postoperative disturbance of consciousness in 4 cases (2.23%) and death in 3cases (1.68%).Conclusions:Sub-frontallongitudinal fissure duramater-keyhole approach is an effective combination of minimally invasive and delicate surgery principle. It is a safe and effective method for the treatment of craniopharyngioma.

Objective:To study the cause of frontal sinusitis and the prevention methods after opened frontal sinus in the trans-frontal approach surgery.Methods:Retrospectively analyze 18 cases of frontal sinusitis secondary to the trans-frontal approach surgery in Sanbo Brain Hospital, Capital Medical University from January 2016 to December 2020. The main symptoms include headache, stuffy nose, skin redness and skin abscess in frontal. The mean history was 3.2 years, all the patients were performed surgical treatment, including 5 patients undergone trans-nasal trans-sphenoid sinus operation, 14 patients undergone debridement surgery following the original surgical approach. The cause of frontal sinusitis were analyzed statistically.Results:All the patients were totally cured post operation, the standard included no frontal sinus inflammation symptoms, no imaging characters of frontal sinusitis.Conclusions:Proper management of opened frontal sinus in trans-frontal approach is very important for preventing the frontal sinusitis. Completely remove the frontal sinus mucosa, avoid using bone wax, chemical glue stuffing, which can effectively prevent the frontal sinusitis.

神经外科医生应专注于手术，且能"跳出少陵窠臼外"，借助新工具新理念，促使新技术不断地衍生，以使手术愈加精准和微创。额底纵裂入路做为切除颅咽管瘤的主流手术入路，也在不断地改进发展。

目的 探讨男性巨大垂体泌乳素(PRL)腺瘤的临床特征及手术治疗效果.方法 回顾性分析2018年7月～2019年7月手术治疗的8例男性巨大垂体PRL腺瘤的临床资料.结果 术前血清PRL水平在50.8～9 852 ng/ml,平均3 505.6 ng/ml.肿瘤全切除3例,近全切除4例,大部分切除1例.术后血清PRL恢复正常4例.8例术后症状均有好转.结论 男性巨大垂体PRL腺瘤,可首选溴隐亭治疗,若颅内压增高症状明显,可选择手术治疗.术中尽可能全切除肿瘤,保护正常垂体,术后酌情继续应用溴隐亭治疗或放疗.

Objective:To investigate the clinical and neuroimaging features of atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system and the survival prognosis in different locations.Methods:The clinical data of 27 patients with AT/RT confirmed by biopsy or postoperative pathology in Sanbo Brain Hospital of Capital Medical University from October 2012 to September 2020 were collected, including 17 males and 10 females, aged (7.6±10.0) years, ranging from 0.2 to 39.0 years old.The clinical features and the results of the first preoperative imaging examination were retrospectively analyzed.The patients were divided into supratentorial, infratentorial and trans supratentorial and infratentorial groups according to the location.The survival time of the three groups was compared by Kaplan-Meier survival curve.Results:All patients presented with headache, including 12 cases with vomiting.There were 12 cases of supratentorial, 10 cases of infratentorial and 5 cases of supratentorial and infratentorial.There were 20 cases of cystic degeneration, 7 cases of calcification, 6 cases of hemorrhage and 13 cases of peritumoral edema.The median survival time of patients with infratentorial AT/RT was longer than that of patients with supratentorial and transtentorial AT/RT (χ 2=7.353, P=0.025). Conclusion:Central nervous system AT/RT is easy to occur in young children, and the survival time of AT/RT patients is longer.

Objective:To explore the surgical method and clinical effect of craniopharyngioma resection through the cockscomb approach.Methods:A retrospective case-control study was conducted from December 2010 to May 2018.The clinical data, imaging data and follow-up data of 101 patients with craniopharyngioma confirmed by pathology by pathology after resection of the frontal floor longitudinal fissure through the crow′s comb approach were performed in Department of Neurosurgery, Sanbo Brain Hospital Capital Medical University.The total tumor resection rate, mortality and complications were analyzed by statistical methods.Results:Through this approach, the total resection rate of tumor was 90.1%(91/101), and the mortality was 1.9%(2/101). The most common postoperative complications were disturbance of sodium metabolism (54.5%(55/101)).Conclusion:Sub-frontal longitudinal fissure crista galli approach can safely and thoroughly remove the tumor and avoid the injury of the hypothalamus and its surrounding tissues as far as possible.

目的:研究鼻内镜手术用于治疗慢性鼻-鼻窦炎的临床疗效和影响疗效的影响因素.方法:选取我院2017年3月至2018年10月期间收治的56例慢性鼻-鼻窦炎患者作为研究对象,根据治疗方式不同将其定义为两组,探究组采用鼻内镜手术进行治疗,一般组患者采用非手术治疗,对比两组患者临床疗效.结果:探究组患者临床治疗有效率高于一般组,两组间差异有统计学意义(P<0.05);探究组患者临床复发率低于一般组,两组间差异有统计学意义(P<0.05).结论:鼻内镜手术用于治疗鼻-鼻窦炎临床疗效显著,疾病分型、手术操作技术以及治疗药物都是影响鼻-鼻窦炎患者临床疗效影响因素.

目的 探讨儿童和青春期脑膜瘤的诊断、治疗及预后.方法 回顾性分析2013年4月至2018年4月收治的16例儿童和青春期脑膜瘤患者的临床资料.结果 肿瘤位于颅底区域8例,镰旁窦旁2例,脑室4例,其他区域2例.单发肿瘤者11例,多发肿瘤者5例.根据肿瘤部位选择手术入路,肿瘤全切除13例,未切除3例.WHOⅠ级均为纤维型脑膜瘤;WHOⅡ级8例,其中内皮型1例、纤维型1例、非典型4例、脊索型2例;WHOⅢ级均为横纹肌型.预后:未复发2例,复发12例,死亡2例.结论 儿童和青春期脑膜瘤临床少见,全切除是改善预后的重要手段;术后放疗根据切除程度及肿瘤级别进行选择.

鼻中隔偏曲是鼻中隔与中线偏离,出现局部突起,导致鼻腔功能障碍.变应性鼻炎也称为过敏性鼻炎,是鼻腔黏膜变应性疾病,是一种临床常见的耳鼻咽喉科疾病[1].变应性鼻炎是鼻黏膜慢性炎症反应性疾病,特应性个体接触过敏原,释放IgE介导的介质,并且多种免疫活性细胞因子等共同参与,患者的临床表现主要包括水样涕、阵发性喷嚏、鼻塞等.变应性鼻炎发病率高,并且迁延难愈,容易反复发作[2].变应性鼻炎容易合并鼻中隔偏曲,会增加治疗的难度.过去多采用药物治疗,但停药后容易反复.手术治疗受到关注.为探究手术治疗合并鼻中隔偏曲变应性鼻炎患者的临床效果,特选取我院120例患者进行研究,现报告如下.

目的 探讨后颅窝小脑幕切迹型脑膜瘤的手术入路选择,并探讨颞下经小脑幕入路的应用价值.方法 回顾性分析我院2015年8月～2018年7月共14例主体向后颅窝扩展的小脑幕切迹型脑膜瘤患者的临床资料,术前均行头颅CT、MRI(平扫+增强)检查,根据影像学表现,分析该型肿瘤的手术入路选择,最终均采用颞下经小脑幕入路进行肿瘤切除.结果 术后MRI显示Simpson Ⅰ级切除12例,SimpsonⅡ级切除2例,术后14例患者症状均出现消失或不同程度的改善,均未出现严重并发症,1例患者出现头晕、走路不稳,术后7d逐渐消失,1例患者出现术侧听力轻度下降,术后9d恢复正常.经过3个月～3年随访,所有病例均无复发.结论 对主体向后颅窝扩展的小脑幕切迹型脑膜瘤,采用颞下入路能快速断掉肿瘤基底,控制肿瘤出血,且肿瘤全切率较高,并发症较少,是一种较好的术式选择.

目的 分析评价鼻内镜下双极电凝止血对顽固性鼻出血患者应激指标的影响研究.方法 在此次研究过程中,一共纳入顽固性鼻出血患者72例,入选地点为我院,入选时间为2017年1月-2018年1月;进一步按随机数字表法分成两组,每组平均为36例;其中,对照组患者采取单纯堵塞方法治疗,观察组患者采取鼻内镜下双极电凝止血方法治疗,进一步比较2组疗效及应激指标水平变化情况.结果 观察组、对照组治疗总有效率分别为94.44％、69.44％,观察组显著高于对照组(P<0.05).结论 在临床中,针对顽固性鼻出血患者,采取鼻内镜下双极电凝止血医治方案效果明显,能够促进应激指标水平的改善,同时使术后并发症发生率降低.

目的 对比分析三种手术方式治疗腺样体肥大,其中低温等离子融切术的疗效和安全性.方法 回顾性分析2008年1月至2019年2月我院收治的137例由于腺样体肥大引起OSAHS的患儿,A组32例接受鼻内窥镜下腺样体刮除术+经鼻动力系统切除腺样体,B组61例接受鼻内窥镜下等离子辅助下行腺样体融切术,C组44例接受鼻内窥镜下腺样体刮除术+经鼻咬除腺样体手术.就三组的疗效、并发症、安全性进行对比.结果 总有效率:A组:100.0％B组:100.0％C组:95.4％,差异有统计学意义(P<0.05).术中出血量:B组(8.09±6.85)ml<A组(26.56±12.01)ml及C组(29.43±11.97)ml,差异有统计学意义(P<0.05).手术时间:B组短于A组及C组,具有统计学意义(P<0.05).术后第一天咽痛程度:B组明显优于A组与C组(P<0.05).鼻腔粘连率:B组优于A组与C组(P<0.05).结论 腺样体肥大患儿治疗中鼻内镜下低温等离子辅助腺样体融切术治疗临床疗效理想,出血量少,更加安全可靠.

目的 探究痉挛型脑瘫患儿实施显微神经外科手术治疗后获得的临床效果.方法 本次研究于2015年1月至2017年-11月期间收集31例痉挛型脑瘫患儿参与,所有患儿均行显微神经外科手术治疗,术后行24个月随访,对本组患儿临床疗效进行分析.结果 术后共27例患儿痉挛状况得到良好缓解,占比(87.09％),1例并发肌无力,占比(3.23％).结论 痉挛型脑瘫患儿采取显微神经外科手术治疗可获得良好的效果,痉挛症状有明显的改善,因此可在临床中对该手术方式进行推广.

目的 探究颅内肿瘤患者采取锁孔手术治疗的效果.方法 本次于2017年1月至2017年12月收集我院神经外科颅内肿瘤患者70例参与研究,根据随机数字表法将其分为对照组与观察组,每组35例.对照组患者行常规开颅手术,观察组则行锁孔手术.对比两组患者临床疗效以及手术指标.结果 观察组患者手术时间、手术出血量均显著低于对照组,数据差异有统计学意义(P<0.05);观察组、患者致残率以及病死率显著低于对照组,治愈率则显著高于对照组,数据差异有统计学意义(P<0.05).结论 颅内肿瘤患者实施神经外科锁孔手术治疗效果理想,值得推广.

Objective To evaluate the feasibility and safety of supratotal resection of frontal or temporal lobe glioblastoma by the postoperative cognitive function and overall survival. Methods The clinical data of patients with frontal or temporal lobe glioblastoma and treated from January 2010 to January 2015 were analyzed retrospectively.The patients underwent supratotal resection of glioblastoma after March 2012(trial group, 33 cases). Before March 2012, the patients underwent total resection of glioblastoma(control group,28 cases).The Montreal cognitive assessment(MoCA)scores before operation and the 7th day after operation were used to evaluate the security, while overall survival was used to evaluate the feasibility. Variance analysis of repeated measurement quantitative data was used for the statistical analysis of the results. Multivariate Cox model was used as a method to estimate the independent association of a variable set with overall survival. Survival time observation was plotted by the Kaplan-Meier analysis,starting from the data of surgery.Results The MoCA scores in control group at the 7th day after operation were significantly lower than those before operation:(25.39 ± 3.04)scores vs.(26.67 ± 2.19)scores, t = 4.446,P < 0.05. The MoCA scores in trial group at the 7th day after operation were significantly lower than those before operation:(21.93 ± 4.46)scores vs.(25.39 ± 3.04) scores,t = 3.485,P < 0.05. The survival analysis of Kaplan-Meier method showed that age, surgical method and preoperative KPS scores were statistically significant (P < 0.05). Cox regression analysis showed that the choice of surgical method and preoperative KPS was 0.286 and 0.965,respectively.The risk of death in control group was 0.286 times of that in trial group.The survival time of trial group was better than that of control group.Conclusions The cognitive function of patients with frontal or temporal glioblastoma may have a certain degree of decline after tumor resection or supratotal resection.Supratotal resection could represent a promising strategy that can impact on outcome in glioblastoma patients. Supratotal resection could be a factor influencing survival.

OBJECTIVE:A retrospective review of the surgical outcome for patients with craniopharyngioma (CP) treated in a single neurosurgical center with surgical resection using visualization to ensure hypothalamic preservation.METHODS:The study included 1054 patients. Before 2003, a pterional cranial approach was preferred for 78% of patients; after 2004, the unifrontal basal interhemispheric approach was performed in 79.1% of patients.RESULTS:Complete tumor resection was achieved in 89.6% of patients; vision improved in 47.1% of patients who had preoperative vision impairment. However, diabetes insipidus worsened in 70.4% of patients and new-onset diabetes insipidus occurred in 29.7% of the remaining patients. Pituitary stalk preservation occurred in 48.9% of cases. There were 89.6% of patients with total tumor removal; 13.3% of patients showed tumor recurrence within an average of 2.8 years. Of 69 follow-up patients with a subtotal or partial resection, 94.2% showed tumor recurrence within an average of 4.3 months. Of the total patients, 82.3% fully recovered.CONCLUSIONS:This study has shown that radical surgical resection of CP using microsurgical excision can be effective with a good patient outcome without more limitations on each individual tumor of distinct features despite the impact of recent endoscopic techniques on CP surgery. The surgical approach depends on a direct and wider visualization of CP located in the midline with preserving hypothalamic structures by identifying some hypothalamic landmark structures. After surgery, most patients can resume their normal activities even after aggressive tumor removal, although patients require postoperative hormonal replacement.