Adjacent epidermoid cyst and primary central nervous system lymphoma: case report

BACKGROUND: Except for cases with radiotherapy or phacomatosis, multiple primary brain tumors of different histologic types are rare. The authors report the association of an epidermoid cyst and a primary CNS lymphoma. CASE DESCRIPTION: A 66-year-old man with a 20-year history of gait disturbance was admitted because of recently worsening symptoms. Magnetic resonance imaging showed an abnormal mass in the posterior fossa. The mass lesion had the appearance of a cystic tumor with a large mural nodule. Gross total removal of the tumor was performed. Histologic examination disclosed the cystic portion to be an epidermoid, whereas the nodular portion exhibited the histologic and immunohistochemical features of a malignant lymphoma. The lymphoma cells were shown to harbor EBV by in situ hybridization. CONCLUSIONS: To our knowledge, this is the first report of the coexistence of an epidermoid cyst and primary CNS lymphoma. When the lesions are adjacent, a definite preoperative diagnosis can be difficult. Epstein-Barr virus-associated CNS lymphoma is rare among the immunologically normal population. The possible pathogenesis for the coexistence of these 2 tumors is discussed.     

The different faces of central nervous system metastases

Cerebral metastases are the commonest central nervous system tumors. The MR assessment should include T1-weighted images with and without enhancement and T2/FLAIR images. They usually appear as multiple lesions with nodular or annular enhancement and are surrounded by edema. They are hypervascularized and have no restriction of their diffusion coefficient in their necrotic area and contain lipids on 1H spectroscopy. Metastases can be distinguished from primary tumors by the lack of malignant cell infiltration around the tumor. Stereotactic radiotherapy may temporarily increase tumor volume, although this is not of adverse oncological value. Less commonly, spinal disease may be asymptomatic and should be examined by MR.     

Primary malignant lymphoma of the central nervous system

The occurrence of primary malignant lymphomas of the central nervous system in a population of approximately 1 million people has been analyzed retrospectively. Over a 12-year period, about 1000 tumors of the central nervous system were registered. Among these, 22 were primary malignant lymphomas; incidence, 1.83 per million per year. Thirteen of these tumors were of high-grade malignancy and nine were of low-grade malignancy (Kiel classification of non-Hodgkin lymphomas). The majority of the tumors were B-cell lymphomas, and there were no Hodgkin lymphomas. Neither spinal nor meningeal lymphomas occurred. Nine patients were treated only surgically, whereas 11 received postoperative irradiation or chemotherapy, or both. Of the latter patients, six are alive and well at the time of writing. The importance of making a correct diagnosis preoperatively is stressed, inasmuch as radical operation is unfavorable for these patients. A better prognosis is obtained with combinations of irradiation and chemotherapy.     

小胶质细胞活化对中枢神经系统功能的影响

背景小胶质细胞（microglia,MG）是中枢神经系统（central nervous system,cNs）中一种重要的免疫细胞,MG的活化可对CNS功能产生重要影响。目的通过探讨活化状态的MG生物学特性和临床应用前景,总结出关于MG活化有价值的研究方向。内容对MG细胞活化状态的生物学特性进行探讨,并总结在何种情况下MG活化发挥神经损伤作用,在何种情况发挥神经保护作用及MG活化的可能临床应用及面临问题。趋势MG的活化对CNS既可产生保护作用,又可产生损伤作用,如何通过调节MG的活化状态,趋利避害,是今后的研究方向。     

Solitary fibrous tumor of central nervous system masquerading as meninigioma: Report of a rare case

IntroductionSolitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin that occurs most often in the visceral pleura. It rarely occurs in the central nervous system (CNS). If they occur intracranially, they are extra-axially located and develop from the meninges.Case ReportHerein, we present a case of SFT in a 45 years old female. MR imaging showed isointense to hypointense signal intensity, and marked enhancement. Microscopically, the tumors showed characteristic “patternless-pattern” architecture. Elongated tumor cells formed fascicles alternating with hypocellular densely collagenous stroma. Immunohistochemistry for STAT6, CD34, BCL2 and CD99 favours the definitive diagnosis of SFT.ConclusionIn general, complete surgical resection may offer the best chance of a favourable clinical outcome. Their biological features remain largely unknown. Hence, the clinical management and prognosis is often challenging due to the lack of comprehensive data.     

原发性中枢神经系统淋巴瘤MRI表现

目的探讨原发性中枢神经系统淋巴瘤(PCNSL)的MRI表现。方法回顾性分析29例接受MR平扫+增强检查,且经手术病理证实,具有完整临床资料的PCNSL病变的MRI表现,观察病灶的部位、大小、形态、边缘轮廓、瘤周水肿、占位效应、MRI平扫信号特点,重点观察增强后病灶的典型及非典型强化特点。结果 29例中单发者16例,多发者12例(30个病灶),共46个病灶,弥漫浸润型1例。肿瘤好发于胼胝体、基底节区等深部脑组织(35/46,76.09%)。瘤周水肿多为中度(15/46,32.61%)或重度(27/46,58.70%),未见出血及钙化。肿瘤T1WI多呈等低信号(30/46,65.22%)或等信号(14/46,30.43%),T2WI多呈等信号(14/46,30.43%)或等高信号(29/46,63.04%),DWI多为高信号(42/46,91.30%)。增强扫描表现出多种强化方式:典型强化方式包括均匀一致团块状或结节状强化、"裂隙征"、"蝶翼征"、"皮层下刻痕征"、以及"卫星灶征";不典型强化方式包括环形强化、"开环样"强化、4脑室匍匐贴壁生长病灶,沿软脑膜弥漫分布粟粒结节样病灶。结论 PCNSL的MRI表现具有一定的特征性,对PCNSL的诊断及鉴别诊断具有重要的价值,但确诊仍有赖于病理。     

磁共振弥散张量成像在中枢神经系统中的应用

DTI是基于DWI的MRI新技术,不仅能反映脑组织的细微结构,还可通过纤维示踪技术显示白质纤维束。DTI对研究脑的发育、成熟及老化过程及诊断许多中枢神经系统（CNS）疾病具有重要意义。本文就DTI在CNS中的应用予以综述。     

Breast cancer subtypes and outcomes of central nervous system metastases

Central nervous system (CNS) metastases are detected in up to one third of patients with advanced breast cancer, but their incidence and outcomes by breast cancer subtypes are not precisely documented. Herein, we retrospectively analyzed clinicopathologic data of 259 breast cancer patients with CNS metastases to evaluate the association between breast cancer subtypes and CNS metastasis. The patient groups were classified according to their hormone receptor status and HER-2 expression. Median follow-up time among the patients was 42 months and median survival after CNS metastasis detection was 7.8 months. In HER-2 overexpressing group, median time period between the diagnosis of breast cancer and the detection of CNS metastasis (15.9 months) was significantly shorter compared to the other groups (p = 0.01). The triple negative group had the shortest median survival time after CNS metastasis (6.6 months), although statistically not significant (p = 0.3). In multivariate Cox regression analyses, having solitary CNS metastasis (HR 0.4, 95% CI; 0.2–0.7, p = 0.004), and receiving chemotherapy after CNS metastasis (HR 0.4, 95% CI; 0.287–0.772, p = 0.003) were independent prognostic factors for increasing survival after CNS metastasis. In conclusion, new and effective treatment strategies are required for breast carcinoma patients with brain metastasis considering the positive effect of the treatment on survival.     

原发性中枢神经系统淋巴瘤的诊断和治疗

目的探讨原发性中枢神经系统淋巴瘤（PCNSL）的临床特点,提高对PCNSL的认识。方法回顾性分析43例PCNSL患者的临床表现、实验室检查、影像学特点、组织病理类型以及治疗方法。结果单发病灶36例,多发病灶7例,共手术治疗47次。首发症状为颅内压增高22例（51．2％）,肢体偏瘫、抽搐、语言障碍16例（37．2％）,记忆力下降、意识情感障碍10例（23．3％）。脑脊液蛋白含量高于正常者66．7％（8／12）。B细胞型淋巴瘤40例（97．6％）,T细胞型1例（2．4％）。Ki-67阳性表达指数42．8％±23．3％。结论CT、MRI及脑脊液检查是术前诊断PCNSL的重要方法,外周血淋巴细胞增高不能作为诊断PCNSL的依据。手术在于缓解症状和确定病变的病理类型,术后化疗加全脑放疗是治疗PCNSL的有效方法。     

线粒体跨细胞转运参与中枢神经系统疾病研究进展

线粒体是细胞氧化磷酸化及合成ATP的主要场所,在维持钙稳态、调节活性氧自由基产生、能量平衡与代谢、诱导程序性细胞死亡中均发挥重要作用。越来越多的证据提示线粒体可在细胞间发生转移,对机体产生保护或有害的影响。多种转运方式介导线粒体的跨细胞转运,然而深入的转运机制仍未完全阐明。目前发现线粒体跨细胞转运在多种中枢神经系统疾病损伤修复中具有重要意义。文章主要就线粒体功能、线粒体跨细胞转运及其转运机制、线粒体跨细胞转运与中枢神经系统疾病进行概述。     

异基因造血干细胞移植后并发中枢神经系统急性移植物抗宿主病1例并文献复习

目的观察急性白血病患者接受异基因造血干细胞移植（allogeneic hemotopoietic stem cell transplantation,allo-HSCT）后并发中枢神经系统急性移植物抗宿主病（aGVHD）的临床特点。方法回顾性分析1例急性髓细胞白血病（acute myeloid leukemia,AML）M5b型患者于allo-HSCT后并发中枢神经系统aGVHD的临床资料。结果患者接受allo-HSCT后40d突发癫,根据头颅磁共振成像（MRI）表现以及相关科室会诊意见,考虑中枢神经系统aGVHD,经大剂量甲泼尼龙冲击治疗后病情好转,1个月后复查头颅MRI示病灶基本消失。结论急性白血病接受allo-HSCT后发生中枢神经系统aGVHD较少见,临床表现缺乏特异性,加强监测排除其他疾病,确诊后及早使用肾上腺皮质激素（激素）,可使患者获得较好的转归。     

Hemangiopericytoma in the central nervous system. A study of eight cases

Most hemangiopericytomas (HPC) are located in the musculoskeletal system and the skin, while the location in the central nervous system (CNS) is rare. The latter represents 2 to 4% in large series of meningeal tumors, thus accounting for less than 1% of all CNS tumors. In the central nervous system, tumors with a hemangiopericytomatous histolopathological pattern can be either hemangiopericytomas or solitary fibrous tumors. CNS-HPCs have a relentless tendency for local recurrence and metastases outside the CNS. Metastasis can also appear many years after adequate treatment of the primary tumor. We present a pathological study of eight patients with CNS-HPC and compare our results with corresponding published data. The CNS-HPC group consisted of three males and five females with a mean age of 36.75 years. The tumors were supratentorial in four cases, infratentorial in two cases, tentorial in one case and located in the spinal cord in the last one. Histologically, CNS-HPCs were similar to their soft tissue counterparts. One case demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism with infiltration of the cerebellum. All patients underwent surgery with gross-total resection in all cases. No patients received postoperative radiation therapy. Only four patients recurred locally after six, seven and eight months, and five years. Our study presents the pathological features of CNS-HPC as a distinct entity from both meningioma and solitary fibrous tumors. A comparative review of literature with our results is discussed.     

细菌性中枢神经系统感染的实验室诊断研究进展

细菌性中枢神经系统感染是常见且严重的颅内感染性疾病,其早期实验室诊断对治疗和预后有重要作用。细菌性中枢神经系统感染的实验室诊断由脑脊液常规和生化等传统检测,逐渐发展为PCR技术、核酸杂交、16S r RNA测序以及基因芯片技术的多方法、多指标联合检测和多学科合作,促进了该病早期、快速的实验室诊断发展。本文就细菌性中枢神经系统感染的流行病学、常见致病菌及实验室诊断等方面进行了综述。     

中枢神经系统胃动素的分布及其对胃肠动力调控的机制

背景 研究表明胃动素为一种脑肠肽,除胃肠道和血浆以外,在中枢神经系统也有广泛分布,于多水平参与机体胃肠及其他功能的调控,并发挥重要作用. 目的 探讨中枢胃动素的分布及对胃肠动力的调控机制. 内容 综述胃动素的分子生物学特性、信号转导通路以及在中枢神经系统的分布和作用等. 趋向 中枢胃动素的分布及其对胃肠动力的作用机制正在成为新的研究热点,特别是各种疼痛诱发的中枢及外周水平胃动素表达的变化及其信号转导通路值得我们深入研究.     

The central nervous system in childhood chronic kidney disease

Neurodevelopmental deficits in pediatric and adult survivors of childhood onset chronic kidney disease (CKD) have been documented for many years. This paper reviews the available literature on central nervous system involvement incurred in childhood CKD. The studies reviewed include recent work in neuroimaging, electrophysiology, and neuropsychology, along with commentary on school functioning and long-term outcomes. The paper concludes with suggestions for monitoring the neurodevelopmental status and pursuing appropriate early interventions for children with CKD.     

MRI评估胎儿神经系统畸形

目的探讨MRI诊断胎儿中枢神经系统畸形的价值及其应用指征。方法回顾性分析450例中枢神经系统畸形胎儿的超声和MRI资料,与产后诊断或尸体检查结果进行对照。结果 450例患者共检出神经系统病变594处,包括单纯侧脑室增宽99处、脑积水21处、小脑延髓池增宽59处、Dandy-Walker畸形/小脑发育不良45处、胼胝体发育不良/缺如67处、透明隔增宽6处、脑白质软化15处、室管膜下囊肿/出血30处、颅内出血26处、颅内实质性病变14处、蛛网膜囊肿60处、孔洞脑13处、脑裂14处、积水型无脑畸形3处、脑水肿1处、平滑脑4处、脑组织发育不良3处、全前脑20处、小头畸形8处、头形异常3处、开放性神经管缺陷68处、脑血管畸形5处、透明隔缺如2处、颅底凹陷症1处、枕骨大孔窄1处、囊性松果体1处、脉络丛病变5处。超声正确诊断487处,准确率81.99%(487/594),MRI正确诊断551处,准确率92.76%(551/594)。结论对于胎儿神经系统各种病变,超声和MRI各有优劣,明确MRI应用指征有助于指导合理地选择产前MRI,从而明确诊断。     

基于SEER数据库的原发性中枢神经系统淋巴瘤不同治疗方法的疗效分析

目的探讨原发性中枢神经系统淋巴瘤(PCNSL)患者的预后因素及不同治疗方法的临床效果。方法回顾性分析1975年1月至2016年12月美国SEER数据库中4812例PCNSL患者的临床资料。其中男性2831例,女性1981例,男女比例为1.4∶1.0;发病年龄<60岁者2236例(46.47%),60~<75岁者1718例(35.70%),≥75岁者858例(17.83%);幕上肿瘤2417例(50.23%),幕下肿瘤299例(6.21%),脑内多发肿瘤554例(11.51%),其他或未指明部位的脑部肿瘤1542例(32.04%);弥漫大B细胞淋巴瘤(DLBCL)3513例(73.00%),非DLBCL 234例(4.86%),其他或未指明类型的淋巴瘤1065例(22.13%);治疗方式为单独活检的2010例(41.77%),单独部分切除61例(1.27%),单独完全切除54例(1.12%),活检+化疗2384例(49.54%),部分切除+化疗159例(3.30%),完全切除+化疗144例(2.99%)。采用单因素及多因素Cox回归模型分析影响患者总体生存的预后因素;采用Fine-Gray检验与竞争风险模型研究影响患者肿瘤特异性生存的预后因素;生存分析采用Kaplan-Meier法,并通过Log-rank进行比较。结果单因素及多因素Cox风险回归模型分析结果显示,影响PCNSL患者总体生存的独立预后因素包括年龄、种族、婚姻状态、肿瘤部位、病理学亚型、手术、化疗、合并其他恶性肿瘤、合并HIV感染等。Fine-Gray检验与竞争风险模型分析结果显示,影响患者肿瘤特异生存的独立预后因素包括年龄、种族、婚姻状态、肿瘤部位、病理学亚型、手术方式、化疗、合并其他恶性肿瘤、合并HIV感染,而性别与放疗均与肿瘤特异生存无明显相关性。与活检相比,PCNSL患者可能从手术切除中获益(部分切除:HR=0.805,95%CI:0.656~0.989,P=0.04;完全切除:HR=0.521,95%CI:0.414~0.656,P<0.01)。Kaplan-Meier生存分析结果显示,活检+化疗组中位生存时间为28个月(95%CI:24.497~31.503),单独活检组为2个月(95%CI:1.756~2.244),单独部分切除组为2个月(95%CI:1.410~2.590),单独完全切除组为19个月(95%CI:0~39.311),部分切除+化疗组为67个月(95%CI:46.187~87.813),完全切除+化疗组为84个月(95%CI:57.448~110.552),使用不同治疗方法的患者的中位生存时间的差异有统计学意义(P<0.01)。结论手术切除可能改善部分PCNSL患者的预后。化疗可能使肿瘤完全切除或部分切除患者的肿瘤特异生存时间延长。     

Intracerebral schwannoma: case report and review

A case of intraparenchymal schwannoma of the brain in a 4-year-old girl is presented. Most of the small number of intracranial schwannomas unassociated with cranial nerves, reported have occurred in children or young adults. There is no male or female predominance. Most of the parenchymatous schwannomas are located supratentorially. The presence of a cyst together with the tumor appears to be characteristic of intraparenchymal schwannoma of the brain.     

Maintenance of cell volume in the central nervous system

Maintenance of the ionic and osmotic composition and volume of intra- and extracellular fluids in the brain is crucial for normal functioning of the central nervous system (CNS). Osmoregulation in the CNS is mediated by solute and water transport across the blood-brain barrier, choroid plexus and plasma membrane of glial cells and neurons. Despite its clinical and physiological significance, however, little is known about the underlying cellular and molecular mechanisms by which CNS osmotic and ionic balance is maintained. In this review, I will discuss our current understanding of cell volume regulation in the CNS and how it relates to various disease processes, such as hyponatremia, renal failure and hypernatremia. A detailed understanding of brain osmoregulatory processes represents a fundamental physiological problem and is required for the treatment of numerous disease states, particularly those encountered in the practice of nephrology.     

中枢神经损伤对大鼠股骨骨折愈合影响观察

摘要：[目的] 观察中枢神经损伤后大鼠股骨骨折愈合速度和骨痂量的变化，分析中枢神经损伤对大鼠股骨骨折愈合速度影响的机制。[方法] 54只雄性Wistar大鼠随机分成3组，（1）脑外伤合并股骨骨折组18只；（2）脊髓损伤合并股骨骨折18只；（3）单纯股骨骨折18只。术后7、14、28d分批处死动物，大体体积测量骨痂，X线片评分，并做HE染色比较。[结果] 脑外伤组，脊髓损伤组形成骨痂体积、X线片评分高于单纯股骨骨折组，差异有显著性（P〈0．05）。HE染色结果显示脑外伤组、脊髓损伤组骨折愈合速度较单纯骨折组加快。[结论] 中枢神经损伤后大鼠骨折愈合加速，骨痂量增多，中枢神经损伤后引起骨痂量和骨折愈合速度的改变，表明中枢神经损伤对骨折愈合有促进作用。