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.     

颅内实质性血管母细胞瘤的显微手术治疗

目的 探讨颅内实性血管母细胞瘤的显微手术治疗方法及疗效。方法 回顾性分析2017年4月至2022年4月手术治疗的16例颅内实性血管母细胞瘤的临床资料。结果 3例予以术前栓塞。12例单发肿瘤均全切除;4例多发肿瘤未全切除。1例术后脑积水行脑室-腹腔分流术后好转,1例死亡,1例术后伤口积液再次行清创手术治疗。术后3个月,未见肿瘤复发,症状明显改善。结论 颅内实性血管母细胞瘤的准确诊断及严密的术前准备对手术切除的成功有重要意义,术前栓塞可明显减少手术并发症及手术风险。     

颅内实性血管母细胞瘤的MRI表现与病理基础

目的探讨颅内实性血管母细胞瘤的MRI特征及病理特点。方法回顾分析10例经手术和病理证实的实性血管母细胞瘤的影像资料,对其MRI特点进行探讨。结果肿瘤位于小脑半球8例,眼眶内1例,第四脑室内1例,影像表现为边界清楚的实性软组织肿块;病变T1WI呈等、稍低信号混杂,T2WI呈等、高信号混杂,占位效应明显而水肿轻;肿瘤内部信号欠均匀;瘤内及瘤周见多发异常血管影,增强扫描实性肿瘤均非常显著强化。结论实性血管母细胞瘤发病率很低,但其MR表现具有一定的特征性,MRI检查有利于肿瘤术前定位及定性诊断。     

后颅窝血管母细胞瘤的显微手术治疗

目的 探讨后颅窝血管母细胞瘤的诊断及手术治疗方法。方法 回顾性分析我科2003—2013年收治的37例经手术及病理证实的后颅窝血管母细胞瘤。结果 发病年龄10~62岁,囊实性28例,实质性9例,所有病例术前均行MRI检查。囊实性肿瘤全切除28例,无死亡病例;实质性肿瘤全切8例,部分切除1例,死亡1例。结论 显微外科手术是后颅窝血管母细胞瘤最重要的治疗手段。     

幕下实性血管母细胞瘤的MRI诊断及鉴别诊断

目的探讨幕下实性血管母细胞瘤的MRI诊断及鉴别诊断,及其MRI特征。方法回顾性分析经病理证实的9例血管母细胞瘤患者的头颅MRI资料。结果本组患者的血管母细胞瘤均为单发,位于左侧小脑半球1例、右侧小脑半球3例、小脑蚓部5例。MRI表现:病灶T_1WI和T_2WI的信号较为混杂; T_1WI以等低信号为主,T_2WI以稍高信号为主,DWI呈低信号;瘤内或瘤周见血管流空影;增强扫描呈明显强化,瘤周中重度水肿。结论幕下血管母细胞瘤的MRI表现有一定的特征性,加深对其认识,可提高术前的诊断率。     

脑毛细胞型星形细胞瘤的MRI诊断分析

目的 总结小脑毛细胞型星形细胞瘤的MRI表现特点.方法 回顾分析16例经手术病理证实的小脑毛细胞星形细胞瘤的MRI表现.结果 16例患者肿瘤位于小脑半球11例(左侧6例,右侧5例),小脑蚓部2例;丘脑2例;脑干1例.MRI检查肿块形态呈类圆形或边缘略不规则形,呈长T1、长T2:信号,其中1例为完全囊性,13例为囊实性,2例为实性,瘤周无水肿或轻度水肿,11例因肿瘤压迫四脑室出现梗阻性脑积水.结论 脑毛细胞星形细胞瘤最好发于小脑,少数见于大脑半球,多表现囊实性,也可为完全囊性或实性.MRI表现具有特征性.但诊断时有时需与血管母细胞瘤、髓母细胞瘤及转移瘤相鉴别.     

伴印戒细胞样细胞分化的中枢神经系统胶质母细胞瘤:病例报告及文献复习

目的 探讨中枢神经系统胶质母细胞瘤的临床表现及病理学特征.方法 回顾分析1例伴印戒细胞样细胞和神经节细胞分化的中枢神经系统胶质母细胞瘤患者的临床表现、组织病理学和免疫表型特点,采用免疫组织化学染色方法诊断与鉴别诊断,并复习相关文献.结果 男性患者,29岁.临床主要表现为头晕、头痛、记忆力减退并渐进性加重,伴双眼视力下降.MRI检查右侧额叶近中线灰白质区占位性病变伴出血.手术中可见肿瘤瘤体主要位于右侧额叶,小部分沿胼胝体浸润至左侧额叶,大小约为4.50 cm×5.00 cm×5.00 cm,与正常脑组织边界不清;肿瘤呈实性、灰红色、质地柔软、血液供应丰富.光学显微镜观察肿瘤主要由胶质分化的细胞组成;肿瘤细胞排列呈实性片状,散在单核或多核瘤巨细胞,可见周围有肿瘤细胞呈假"栅栏"样排列的小灶性坏死及呈肾小球样的间质血管增生;肿瘤细胞具有明显的异型性、细胞核深染以及核分裂象;部分肿瘤细胞呈印戒细胞样细胞和神经节细胞分化特征.免疫组织化学染色肿瘤细胞胞质胶质纤维酸性蛋白、神经微丝蛋白、巢蛋白和蛋白基因产物9.5表达阳性,部分肿瘤细胞胞质嗜铬素A和突触素表达阳性,肿瘤细胞胞核P53蛋白表达阳性,Ki-67抗原标记指数(MIB-1)为20%～30%.结论 中枢神经系统胶质母细胞瘤是恶性程度最高的星形细胞肿瘤,也是中枢神经系统好发的肿瘤,但伴印戒细胞样细胞和神经节细胞分化的胶质母细胞瘤临床少见,好发于成年人,预后不良.     

水通道蛋白1在人成血管母细胞瘤中的表达与分布变化

目的 探讨人成血管母细胞瘤水通道蛋白I(AQP1)的表达变化在肿瘤发展过程中的病理生理意义. 方法 将自1980年至2005年我科收治的26例成血管母细胞瘤患者分为两组:实体肿瘤组与囊性肿瘤组,采用免疫组织化学方法 检测AQPl在成血管母细胞瘤的表达状况,RT-PCR方法 分析成血管母细胞瘤AQP1 mRNA的表达水平变化,激光共聚焦显微镜研究AQP1在血管内皮细胞及肿瘤细胞的表达分布关系.运用Western blot杂交分析AQP1蛋白在成血管母细胞瘤中的表达水平变化. 结果 免疫组织化学结果 显示,与正常脑组织相比,AQP1在成血管母细胞瘤中的表达水平明显升高,差异有统计学意义(P=0.002).在囊性成血管母细胞瘤中,AQP1的表达主要分布在肿瘤间质细胞膜上.在55.6%的实体成血管母细胞瘤中,肿瘤微血管内皮细胞上有AQP1的表达.仅44.4%的实体成血管母细胞瘤肿瘤间质细胞膜上有AQP1的表达.RT-PCR与Western blot杂交分析均表明.成血管母细胞瘤中AQP1 mRNA和蛋白的表达水平升高,且囊性成血管母细胞瘤中AOP1 mRNA和蛋白的表达水平明显高于实体性成血管母细胞瘤. 结论 AQP1表达的异常可能参与了人成血管母细胞瘤的形成与发展过程.同时,AQP1表达、分布的异常可能是人成血管母细胞瘤中瘤囊的形成与发展的重要病理生理机制.     

角回后方皮层入路显微手术切除侧脑室三角区脑膜瘤

目的 探讨侧脑室三角区脑膜瘤的临床特点、诊断以及显微手术治疗方法。方法 回顾性分析2013年9月至2015年12月经角回后方皮层入路显微手术治疗的22例侧脑室三角区脑膜瘤的临床资料。结果 22例肿瘤全切除,无手术死亡病例,术后患者头痛头晕、恶心呕吐症状均明显改善。术后发生命名性失语1例,给予降颅内压治疗后有所改善;出现对侧肢体肌力稍减弱伴有酸麻感1例,出院时恢复良好。术后病理类型:纤维型11例,上皮型3例,混合型1例,血管型1例,具体类型不明确6例。结论 角回后方皮层入路是手术切除侧脑室三角区脑膜瘤较好的入路;术中对供瘤血管的处理及对重要结构的保护是避免术后并发症的关键。     

两家系家族性颅内血管网状细胞瘤的临床特征及诊疗分析

目的 分析2个家系5名家族性血管网状细胞瘤患者的临床表现及诊断治疗方法.方法 湖北省襄阳市中心医院肿瘤科自2005年9月至2011年5月共收治2个家系共5名家族性血管网状细胞瘤患者,回顾性分析患者的临床资料、影像学特征、诊断与治疗方法. 结果 头颅MRI检查显示家系1中3例患者有2例为囊实性肿瘤,1例为实性肿瘤;家系2中2例患者均为囊实性肿瘤.5例患者均存在突变,均可诊断为Von Hippel-Lindau(VHL)病.所有患者均未合并肝脏等其他部位的病变.镜下全切或近全切肿瘤后均行定期随访1年,无复发、无死亡病例. 结论 头颅磁共振检查仍是目前家族性血管网状细胞瘤最主要的诊断及随访方法,手术仍是最主要的治疗手段.     

小脑脑桥角恶性外周神经鞘膜瘤一例并文献复习

目的 结合1例左侧小脑脑桥角(CPA)区的恶性外周神经鞘膜瘤患者的临床资料,探讨罕见部位恶性外周神经鞘膜瘤的发生机制、临床、病理特点及治疗方法.方法 回顾性分析1例左侧CPA区的恶性外周神经鞘膜瘤患者的临床资料.本患者临床表现为无明显诱因下出现反复头晕,左侧颜面部麻木,伴有左侧耳鸣、恶心,无头痛、呕吐.头颅MRI:左侧CPA区见一大小约3.0 cm×2.0 cm ×2.0 cm的团块状等T1长T2信号,其中可见囊变坏死区,周围无明显水肿,增强扫描病灶明显不均匀强化,听神经增粗不明显,可见轻微强化,第四脑室及脑桥明显受压,脑室系统无明显扩大,脑沟裂无明显增宽,中线结构居中.结果 行左侧乙状窦后入路,显微镜下肿瘤切除.病理报告恶性外周神经鞘膜瘤.结论 CPA区的恶性外周神经鞘膜瘤少见,术后病理是诊断的主要依据,予以手术切除肿瘤的同时可合理选择放疗和药物治疗.     

Symptomatic and nonsymptomatic headaches in a general population.

We assessed the lifetime prevalences of headache disorders in a cross-sectional epidemiologic survey of a representative 25- to 64-year-old general population. We classified the headaches on the basis of a clinical interview and a physical and neurologic examination using the operational diagnostic criteria of the International Headache Society. Lifetime prevalence of idiopathic stabbing headache was 2%, of external compression headache 4%, and of cold stimulus headache 15%. Benign cough headache, benign exertional headache, and headache associated with sexual activity each occurred in 1%. Lifetime prevalence of hangover headache was 72%, of fever headache 63%, and of headache associated with disorders of nose or sinuses 15%. Headaches associated with severe structural lesions were rare. External compression headache, fever headache, headache associated with metabolic disorders, and headache associated with disorders of nose or sinuses all showed significant female preponderance. The symptomatic headaches and headaches unassociated with structural lesions were more prevalent among migraineurs. In subjects with tension-type headache, only hangover headache was overrepresented. There was no association between the headache disorders and abnormal routine blood chemistry or arterial hypertension. In women with migraine, however, diastolic blood pressure was significantly higher than in women without migraine.     

Subependymoma of the lateral ventricle presenting with sudden onset

A 52-year-old man presented with sudden onset of severe headache, fever, mental confusion and mild signs of left hemisphere dysfunction. Computed tomographic and magnetic resonance imaging findings suggested a preoperative diagnosis of subependymoma of the left lateral ventricle, which was subsequently confirmed by surgery. The neoplasm could be totally removed and the postoperative clinical course was favourable. The reported case is unusual because symptomatic subependymomas of the lateral ventricle are definitely rare, and almost invariably present with a progressive clinical course over a period of weeks to years.     

侧脑室脑膜瘤的手术治疗

目的探讨侧脑室脑膜瘤的手术入路选择原则及神经导航系统、CUSA（cavitron ultrasonic surgical aspirator，超声外科吸引器）在侧脑室脑膜瘤切除中的作用。方法回顾性分析我院自1999年9月至2007年2月收治的23例侧脑室内脑膜瘤患者临床资料。结果手术全切23例，无手术死亡病例。术前症状改善21例、2例症状改善不明显。9例患者随访5个月至7年，无复发病例。随访病例中，1例术后出现视物变形者3个月后恢复正常，1例术前偏瘫（肌力Ⅰ级）患者2年后肌力明显增加（肌力Ⅲ级），7例无明显不适，正常生活工作。结论侧脑室内脑膜瘤首选显微手术治疗。术前周密计划，个体化选择手术入路加上神经导航系统及CUSA等手术辅助技术设备、工具的应用可以提高肿瘤的全切程度，减少手术并发症的发生。     

第四脑室神经鞘瘤的临床特点及手术治疗并文献复习

目的探讨原发性第四脑室神经鞘瘤的临床特征及手术方法。方法分析1例原发性第四脑室神经鞘瘤手术治疗患者的临床资料,并结合文献分析该病的临床特点和手术方法。结果患者为女性,53岁,表现为剧烈头痛、吞咽困难、共济失调、四肢无力等症状,影像学示第四脑室巨大肿瘤,合并脑积水。手术采用经枕下正中小脑延髓裂入路,肿瘤镜下获得全切除,术后病理诊断为神经鞘瘤。术后随访8个月,未见肿瘤复发。结论尽管脑室内神经鞘瘤罕见,且诊断困难,但该类型肿瘤为良性肿瘤,其边界清楚。因此,手术若能获得有效的全切除,远期预后良好。     

Yolk sac tumor in the fourth ventricle: A case report

The nongerminomatous germ cell tumor occurring in the fourth ventricle is extremely rare. A 9-year-old boy presented with symptoms of obvious headache, projectile vomiting, diplopia and motor weakness. MRI scanning revealed lesions occupying the fourth ventricle, with dual-lateral ventricle expansion after gadolinium administration. Suboccipital craniectomy was performed and the tumor was removed by block resection. Twice intraoperative biopsy specimens revealed fiber vascular tissue and hemangioma. Histological examination revealed that the tumor consisted of loose network structures and Schiller–Duval bodies. Immunohistochemical study showed that the primitive germ cells were positive for placental alkaline phosphatase, alpha-fetoprotein, cytokeratin, vimentin, but negative for glial fibrillary acidic protein. The histological diagnosis was pure yolk sac tumor. Two years after surgery, he has suffered no tumor recurrence. A combination of surgical treatment, radiation and chemotherapy is mandatory.     

Atypical Choroid Plexus Papilloma in an Adult

We present an extremely rare case of the atypical choroid plexus papilloma in an adult which developed at the trigone of right lateral ventricle. A 62-year-old woman presented with the history of intermittent and gradually progressive headache and left side hemiparesis for 6 months. The brain magnetic resonance image showed highly enhanced and well demarcated mass at the trigone of lateral ventricle attached to the choroid plexus. Gross total resection was performed by transcortical approach via the middle temporal gyrus. The tumor was diagnosed as an atypical choroid plexus papilloma. She had no neurologic deficit after the surgery. We report a case of atypical choroid plexus papilloma in adult and introduce newly classified pathologic characteristics of this tumor.     

A lateral ventricular gliosarcoma arising in an ependymoma

OBJECTIVE: We describe a 29-year-old man with gliosarcoma in the lateral ventricle. CASE: The patient presented with headache and impairment of consciousness. Computed tomography and magnetic resonance imaging localized the tumor to the right lateral ventricle and showed heterogeneous enhancement with administration of contrast agents. The tumor was partially removed via a transcallosal approach. Histologic examination disclosed gliosarcoma arising by malignant transformation of an ependymoma. POST-OPERATIVE COURSE: The patient died of tumor progression 78 days after admission, despite intensive radiotherapy and chemotherapy.     

Serial headache drawings by children with migraine: correlation with clinical headache status

Children's artistic self-depictions of headache provide valuable insights into their experience of pain and aid in the diagnostic differentiation of headache types. In a previous study, we compared the clinical diagnosis (gold standard) and artistic diagnosis of headaches in 226 children. In approximately 90% of cases, the drawing predicted the clinical diagnosis of migraine versus nonmigraine headache correctly. In the present study, we explored whether headache drawings correlate with clinical improvement after treatment in children with migraine headaches followed longitudinally. Children seen in the Pediatric Neurology Clinic with the chief complaint of headache were asked to draw a picture of what their headache feels like. On subsequent clinic visits, children with the clinical diagnosis of migraine were asked to draw another picture depicting their current headache. The two drawings were compared to assess whether there was improvement; this "artistic response" was then correlated with the child's clinical status (ie, whether the headaches were improved clinically). One hundred eleven children (66 girls, 45 boys) participated in the study, with a mean interval of 5.3 +/- 2.3 (standard error of the mean) months between the first and second visits. The mean age at the first visit was 11.6 +/- 3.1 years. The raters agreed that serial drawings were both improved or both not improved in 99 of the 111 cases (89%; interrater reliability kappa score of 0.767). Fifty-three children had improvements in their headaches and drawings, 3 children had an improved drawing but no clinical headache improvement, 32 children had no improvement in either their drawing or clinical headaches, and 11 children had improved headaches but no improvement in their drawing. The sensitivity of the drawings for clinical improvement was 0.83, and the specificity was 0.91. The predictive value of an improved headache drawing for an improved clinical response was 0.946. There was no correlation between specific treatment modality and artistic response. We concluded that children's headache drawings are a useful adjunct for the diagnosis of headache type and provide valuable insights into their experience of pain. The present data show that headache drawings can be used longitudinally to provide additional information about the clinical course. The technique is simple, inexpensive, and enjoyable for children and can be applied in a variety of clinical settings.     

半结构性问询在儿童复发性头痛诊断中的运用

目的探讨半结构性问询在儿童复发性头痛诊断中的运用。方法对2007年10月至2010年10月门诊及病房诊治的90例复发性头痛病人进行半结构性问询、随访、全面的体格检查及神经系统检查并辅以必要的辅助检查。结果①90例复发性头痛患者中88例(97.8%)为原发性头痛,63例偏头痛,其中3例合并紧张性头痛,22例为可能的偏头痛,2例癔病性头痛;继发性头痛2例(2%),分别为颅内肿瘤1例、屈光不正1例;②疑诊为窦性头痛的复发性头痛患者经抗生素及中成药治疗鼻窦炎后头痛无缓解;③实验室检查中经颅多普勒超声检查显示的血管痉挛或扩张的部位与头痛部位无相关性,EEG检查多无明显异常。结论偏头痛是复发性头痛的常见原因;慢性鼻窦炎无急性发作不会导致窦性头痛。在复发性头痛诊断中应用结构性问询,并加强随访,既提高了诊断率,又避免了不必要的检查。