儿童侧脑室毛细胞型星形细胞瘤合并NF-11例

目的总结1例儿童侧脑室毛细胞型星形细胞瘤(PA)合并神经纤维瘤病Ⅰ型(NF-1)的诊疗经验。方法回顾性分析1例NF-1儿童侧脑室PA病人的临床资料,采用左侧额部开颅皮质造瘘入路,显微外科切除肿瘤。结果肿瘤次全切除,术后病理证实为PA。术后3个月复查MRI考虑肿瘤复发,随访观察。结论 NF-1儿童侧脑室PA罕见,手术难以全切,术后易复发。     

毛细胞型星形细胞瘤的诊断与治疗

目的分析毛细胞型星形细胞瘤的临床特点及诊治方法。方法回顾性分析解放军总医院1997年1月～2007年1月手术治疗的37例毛细胞型星形细胞瘤患者的临床资料。结果病理显示典型毛细胞型星形细胞瘤30例（81.2%）,黏液型毛细胞型星形细胞瘤7例（18.8%）;28例肿瘤全切,31例平均随访45个月。结论毛细胞型星形细胞瘤的诊断主要依赖MRI检查,显微手术全切肿瘤是本病的主要治疗方法,应在保留功能的前提下尽量切除肿瘤,术后放化疗存在争议,复发的肿瘤可再次手术;应加强对黏液型毛细胞型星形细胞瘤的认识。     

毛细胞型星形细胞瘤的诊断和治疗

目的　探讨毛细胞型星形细胞瘤发病特点、病理学和影像学表现以及治疗方法。方法　 回顾性分析我院近3年来47例经手术病理证实的毛细胞型星形细胞瘤临床资料。结果 47例患者平均发病年龄21岁,20岁以下28例(59.6％)。位于小脑及大脑半球者可分为囊性伴囊壁结节、假囊性伴囊壁结节、实质性3种影像学表现。鞍区、脑干、脊髓等部位多表现为实质性。镜下多可见到大量Rosenthal氏纤维。46例GFAP免疫标记阳性,38例Vim免疫标记阳性。随访20个月,全切组(36例)无肿瘤复发,部分残留组(11例)1例复发。22例术后放疗,随访期内无肿瘤复发,未放疗组1例肿瘤复发。结论 毛细胞型星形细胞瘤有比较典型的影像学和病理学特点,外科全切术是首选的治疗方法。对于未全切病例术后应行放疗。     

儿童小脑毛细胞型星形细胞瘤的诊治(附15例报告)

目的总结儿童小脑毛细胞型星形细胞瘤的临床资料，探讨其诊治特点。方法分析儿童小脑毛细胞型星形细胞瘤15例的影像学、病理学特点及术中所见和预后情况。结果肿瘤全切除13例，次全切或大部切除2例。术后发热2例，均于术后3d内消失；头痛2例，随病情好转而逐渐消失。余病例未出现新的症状和体征。术后随访未见到复发。结论儿童小脑毛细胞型星形细胞瘤有其独特的临床特点，在条件允许的情况下应尽可能切除肿瘤。     

毛细胞型星形细胞瘤诊治分析

目的探讨毛细胞型星形细胞瘤的诊治经验。方法回顾性分析60例经病理证实的毛细胞型星形细胞瘤的临床资料,肿瘤部位:颅后窝39例(小脑蚓部9例,小脑半球29例,脑干1例),脊髓7例,幕上脑叶4例,侧脑室内4例,鞍区6例。病例均接受显微外科手术,根据病变不同部位,选择合适手术入路。结果全切除56例,部分切除4例。部分切除者术后辅助放疗或伽玛刀治疗。随访6个月~10年,全切除者均无复发,部分切除者保持稳定,肿瘤无明显增长。结论毛细胞型星形细胞瘤是最低级别胶质瘤,若能全切除病变,无需辅助治疗,效果良好,预后极佳。对于未能全切除者,辅助放疗或伽玛刀治疗,也能取得良好疗效。     

脊髓髓内星形细胞瘤的显微外科治疗

目的总结脊髓髓内星形细胞瘤的手术经验。方法回顾性分析59例脊髓髓内星形细胞瘤的临床资料,包括影像特征、手术技术、术中电生理监护、病理类型、术后并发症、疗效预后等。均行显微手术,术后辅以放疗。采用McCormick分级和MRI影像评估手术疗效。结果肿瘤全切除8例,近全切除26例,部分切除14例,活检11例。随访3个月~2年,术后近期临床神经功能改善32例,无变化23例,加重4例。肿瘤复发13例,其中胶质母细胞瘤11例,间变型星形细胞瘤2例。结论有明确边界的低级别星形细胞瘤首选显微手术治疗,尽可能全切除肿瘤;为保留脊髓功能,对边界不清的高度恶性胶质瘤可行部分切除或活检,以明确诊断和减压为原则。     

含有血管中心性排列特征的毛细胞型星形细胞瘤:病例报告并文献复习

研究背景毛细胞型星形细胞瘤是常见于小脑的低级别胶质瘤,好发于儿童和青少年。本文报告1例临床罕见、发生于成人幕上的毛细胞型星形细胞瘤病例,其病理形态与典型的毛细胞型星形细胞瘤略有不同:脑组织大部分区域肿瘤细胞围绕玻璃样变性小血管呈单层排列,即所谓的血管中心性生长。本文拟探讨含有血管中心性排列特征的毛细胞型星形细胞瘤的临床、影像学及病理学特征,并讨论其与相关肿瘤的临床病理学鉴别要点,以提高对此类肿瘤的诊断与鉴别诊断能力。方法与结果男性患者,28岁。临床表现为反复发作的癫大发作,抗癫药物治疗无效,脑电图检查未发现典型的癫样放电或局灶性δ波。影像学检查以额叶皮质边界清楚的囊性病变伴壁结节为特征性表现。术中可见肿瘤组织质地较韧,表浅为囊性结构、深部呈实质性结构。光学显微镜下观察肿瘤细胞胞核呈圆形、卵圆形,胞质两端突起细长毛发状,围绕玻璃样变性小血管单层排列,部分区域肿瘤细胞胞核伸展呈梭形,束状致密排列或疏松星网状排列,偶见Rosenthal纤维和嗜酸性颗粒小体形成。肿瘤细胞弥漫表达胶质纤维酸性蛋白、波形蛋白、S-100蛋白、巢蛋白和CD56,局灶性表达Olig2,不表达上皮膜抗原、异柠檬酸脱氢酶1、P53、神经元核抗原、神经微丝蛋白、突触素及CD34,Ki-67抗原标记指数约为1%。病理诊断:左侧额叶含有血管中心性排列特征的毛细胞型星形细胞瘤(WHOⅠ级)。术后未行放射治疗或药物化疗,随访1年余未出现癫发作,肿瘤无复发。结论毛细胞型星形细胞瘤好发于儿童,以小脑多见,发生于成人及幕上者少见,含有血管中心性排列特征的毛细胞型星形细胞瘤形态特殊,诊断时应注意与毛细胞黏液型星形细胞瘤、星形母细胞瘤、血管中心型胶质瘤、皮质室管膜瘤及乳头状型胶质神经元肿瘤相鉴别。     

毛细胞型星形细胞瘤的诊断与治疗

目的 探讨毛细胞型星形细胞瘤的临床诊断和最佳治疗方案。方法 通过头颅CT、MRI和手术探讨此类肿瘤的发病率,常见部位,临床特点,手术疗效,预后与手术切除肿瘤程度以及与术后放疗的关系。结果 毛细胞型星形细胞瘤以20岁以下发病率最高77．4％,以小脑居首92%。临床表现以颅内压增高为主、其次共济失调。影像学CT和MRI没有特征性征象,诊断率50％以上,最后确诊主要依靠病理学。预后与手术切除程度有关,肿瘤全切除术后10年内存活率达95％、部分切除术后达72．7％。结论 (1)毛细胞型星形细胞瘤多发病于青少年,以小脑居首。(2)尽可能全切除肿瘤、术后无需放疗其预后良好,可视为“良性肿瘤”;若不能伞切除的病例,术后可给予放疗亦能达到理想的预后。     

多形性黄色瘤型星型细胞瘤的诊断与治疗

目的探讨多形性黄色瘤型星形细胞瘤病人的临床特征及治疗。方法回顾8例多形性黄色瘤型星形细胞瘤病人的临床资料。病人平均年龄23．7岁，肿瘤均位于大脑半球。以癫痫起病，确诊主要依靠病理学检查。结果肉眼全切1例，显微手术全切4例，大部分切除3例；术后行放疗3例，放、化疗2例。随访10个月～5年，6例病情稳定，癫痫及头痛得到控制；2例复发，其中1例死亡。结论多形性黄色瘤型星形细胞瘤相对良性，癫痫发作是最常见的症状。治疗上应尽量手术全切，对术后有残余、复发或间变者可给予化、放疗。     

儿童第四脑室肿瘤的显微外科治疗(附43例报告)

目的 探讨儿童第四脑室常见肿瘤的诊断和显微手术技巧.方法 回顾性分析2006~2010年手术治疗的43例儿童第四脑室肿瘤患者的临床表现与影像学等临床资料;根据肿瘤与小脑蚓部和第四脑室的关系,设计个体化手术入路切除肿瘤.结果 肿瘤全切除38例,次全切5例(为与第四脑室底粘连紧密者).绝大多数病例无明显并发症或并发症较轻,经治疗后好转.病理结果示髓母细胞瘤27例,室管膜瘤8例,星形细胞瘤7例(其中毛细胞型3例),神经细胞瘤(交界性)1例.髓母细胞及WHOⅢ级的室管膜瘤术后进行了全脑和脊髓放疗,次全切的室管膜瘤和星形细胞瘤进行了局部放疗.结论 儿童第四脑室肿瘤以髓母细胞瘤最多见,其次是室管膜瘤和星形细胞瘤;正确的入路选择和手术技巧是取得良好疗效、减少并发症的关键.     

Responsiveness of progressive optic pathway tumors to cisplatin-based chemotherapy in children

Background  Though the pathology of optic pathway tumor is mostly pilocytic astrocytoma, the benign tumor behaves like malignant tumor because total resection is not feasible. Adjuvant chemotherapy might be a reasonable strategy for management of these low grade tumors which keep growing. We evaluate the responsiveness of optic pathway tumor to cisplatin-based chemotherapy. Methods  Patients with unresectable and progressive optic pathway tumors received conventional chemotherapy including cisplatin, etoposide, and vinblastine were enrolled in this study from 1992 to 2007. Patients treated with radiotherapy previously were excluded. Brain MRI was performed every 3 months to evaluate the objective response to chemotherapy. Results  There are seven girls and nine boys enrolled in this study. The median age at diagnosis was 30 months old (range from 3 months to 11 years old). The median follow-up duration was 81.5 months (range from 24 months to 14.5 years). The pathology showed pilocytic astrocytomas in 11 patients, astrocytoma in one patient, and anaplastic astrocytomas in two patients. The 6-month progression-free survival (PFS) is 100%, 12-month PFS is 81.3%, 3-year PFS is 71.4% and 5-year PFS is 55.5% respectively. The toxicity of the cisplatin-based chemotherapy showed mild bone marrow suppression in 13 patients (81.3%), infection in nine patients (56.3%), gastrointestinal discomfort in seven patients (43.8%), renal insufficiency in two patient (12.5%), cerebral salt wasting syndrome with hyponatremia in one patient (6.25%) and high pitch hearing loss in two patients (12.5%). Conclusion  Cisplatin-based chemotherapy is an effective regimen for control of progressive optic pathway tumors. Ting-Rong Hsu and Kai-Ping Chang contributed equally to this paper.     

AgNOR staining may reflect the growth potential of pilocytic astrocytomas

The value of AgNOR staining as a tumor biological marker was tested in 26 children with pilocytic astrocytomas (20) and fibrillary astrocytomas (6). All patients were surgically treated and then followed up by periodic MRI or CT scans. Follow-up ranged from 8 to 84 months, with a mean of 44 months. AgNOR expression was determined by using semi-automated computer-assisted surface area measurements. AgNOR values ranged from 1.4 to 81.4 μm² per cell, with a mean of 26.6 and a median of 15.2. The median value was taken as a ”cut-off” score separating two groups of patients with low and high AgNOR scores. Of the 13 patients in the low scoring group, 8 had total resections without recurrence, 3 had stable residual tumors, 1 had regressing residual tumor after irradiation and 1 had a recurrence 5 years after neuroradiologically complete resection of a fibrillary astrocytoma. In the group with high AgNOR scores only 2 patients had total resections without recurrence; 5 had stable residual tumors and 6 had residual tumors that showed progression, all within 1 year after surgery. Among the patients with classic juvenile pilocytic astrocytomas of the cerebellum 7 had residual tumor, which progressed in 2 patients, both of whom had high AgNOR scores. Among 7 patients with optic/hypothalamic tumors the 3 with rapidly progressing tumors all had very high AgNOR scores. The determination of AgNOR expression might be helpful in selection of patients with residual tumor after surgery, who may benefit from additional chemotherapy or (stereotactic) radiation therapy. Received: 12 August 1998 Revised: 22 March 1999     

以癫癎为首发症状的低级别星形细胞瘤病人术后癫癎预后分析

目的探讨影响以癫癎为首发症状的低级别星形细胞瘤癫癎预后的因素。方法回顾性分析以癫癎为首发症状的低级别星形细胞瘤病例,经手术切除肿瘤1年后的癫癎症状改善情况,本组研究数据应用SPSS17．0统计软件进行卡方检验和多因素Logistic回归分析。结果术后癫癎预后为改良Engel I级102例（67．5％）,Ⅱ级以上49例（32．5％）。卡方检验显示：术前病人有语言障碍（P=0．038）、术前肿瘤累及岛叶（P=0．010）、肿瘤未全切除（P=0．001）对术后癫癎预后差异有统计学意义。多因素Logistic回归分析：术前病人有语言障碍（P=0．022）、术前肿瘤累及岛叶（P=0．007）及肿瘤未全切除（P=0．002）均为影响术后癫癎发作的独立危险因素。结论术前病人有语言障碍、术前肿瘤累及岛叶及肿瘤未全切除是影响以癫癎为首发症状的低级别星形细胞瘤病人术后癫癎预后的独立危险因素。     

Cerebellar astrocytomas: a 24-year experience

INTRODUCTION: Cerebellar astrocytomas are the most benign tumors of the CNS. Seventy to eighty percent are found in children. METHODS AND RESULTS: We report on 38 children under 18 who had cerebellar astrocytoma in the posterior fossa and were treated by a multidisciplinary team in our Neurosurgical Department from January 1974 to December 1997. We included all patients in whom the histopathological diagnosis was astrocytoma, regardless of malignancy. The diagnostic methods used were pneumoventriculography, cranial X-rays, CT scan, and MRI. All patients were treated surgically. Neither radiotherapy nor chemotherapy was indicated in patients with pilocytic or fibrillary astrocytomas. A greater prevalence was observed in female (25/38; 66%) than in male (13/38; 34%) patients. Histopathological results revealed 27 (71%) pilocytic astrocytomas, 8 (21%) diffuse fibrillary astrocytomas, 1 (2%) anaplastic astrocytoma and 2 (6%) glioblastomas. These tumors were more frequently located in the right cerebellar hemisphere; increased intracranial pressure syndrome was the most frequent form of clinical presentation. Total tumor resection was obtained in 29 (83%) cases and subtotal resection in 9 (17%). In 6 (16%) cases, ventriculoperitoneal shunts were placed to control persistent hydrocephalus after tumor excision. CONCLUSION: The most frequent complication was increased ataxia. The mortality rate was 8.5%.     

成人毛细胞型星形细胞瘤的诊治分析

目的 探讨成人毛细胞型星形细胞瘤（PA）的诊断、显微手术治疗效果。方法 回顾性分析2009年9月至2018年12月新疆医科大学第一附属医院神经外科手术治疗的31例成人PA的临床资料。结果 肿瘤全切除17例,近全切除4例,次全切除6例,部分切除2例,活检2例。术后主要并发症包括脑积水3例（9.7%）、颅内感染4例（12.9%）、脑脊液漏3例（9.7%）。26例术后随访3~93个月;死亡3例（11.5%）;肿瘤复发或进展5例（19.2%）,平均复发时间（25.6±16.9）个月,其中2例再次手术,肿瘤全切除本人随访期间未见肿瘤复发。结论 成人PA临床少见,影像学常不易与其它低级别胶质瘤鉴别,而典型的组织细胞学特点是其确诊依据;肿瘤全切除的本人能获得较满意的治疗效果,放疗为PA的主要辅助治疗方式。     

rCBV变化与VEGF表达对胶质母细胞瘤术后放疗病人生存期的预测研究

目的 探讨相对脑血容量(relative cerebral blood volume,rCBV)变化和血管内皮生长因子(vascular endothelial growth factor,VEGF)表达对胶质母细胞瘤全切手术后辅助放射治疗病人的无进展生存期(PFS)和总生存期(OS)的预测作用.方法 回顾性分析16例外科全切术后辅助放射治疗的胶质母细胞瘤病人的临床资料.在放射治疗前和放射治疗中(累计放射剂量为30 Gy)各进行1次灌注成像检查,计算rCBV值.免疫组化检测VEGF表达.采用Kaplan-Meier生存曲线分析PFS和OS.结果 不同年龄、VEGF表达和rCBV变化的病人,其在PFS的差异具有统计学意义(均P＜0.05).仅有VEGF表达不同的病人在OS的差异具有统计学意义(P＜0.05).结论 VEGF表达和rCBV变化可以作为判定胶质母细胞瘤PFS的预测物.同时,VEGF表达可以作为判定胶质母细胞瘤OS的预测物.     

Long-term survival in children under 3 years of age with low-grade astrocytoma

Objective The purpose of this study is to analyze clinical aspects and disease-free survival (DFS) in children less than 3 years of age diagnosed with low-grade astrocytoma. Methods In a period of 24 years (1980–2004), a total of 43 (5.4%) children were registered with these characteristics. Twenty-three patients had pilocytic astrocytoma, 18 diffused, and 2 mixed. Thirty-one (72.1%) children had incomplete surgical tumor resection and 12 (27.9%) had a complete tumor resection. Twelve (27.9%) patients had cranial radiotherapy and 17 (39.5%) received chemotherapy. Overall survival was recorded in 23 (53%). DFS was 50% at 250 months of follow-up for the whole group. DFS for the supratentorial group was 60% at 250 months, whereas, for the infratentorial, it was 22% at 120 months (p = 0.008). Conclusion The only favorable prognostic pattern was the supratentorial presentation. Radiotherapy and chemotherapy did not alter the outcome.     

Hematoma in a low-grade medullary astrocytoma: report of an unusual case and literature review

We describe the case – to our knowledge unique – of an 8-year-old boy who presented with acute onset of lower cranial nerve palsy and tetraparesis caused by a hematoma in a dorsal exophytic pilocytic astrocytoma of the medulla oblongata. The boy showed near-complete recovery after neurosurgical management in two stages: first, emergency evacuation of the hematoma with tumor biopsy, and second, complete tumor removal 5 months after the initial event. Intraoperative electrophysiological monitoring techniques for the lower cranial nerves are of value in preserving their functional integrity. Ultrasonography is helpful in assessing the extent of tumor removal. Although the pathological diagnosis of a pilocytic astrocytoma would not justify radiotherapy, local field radiotherapy was added mainly because of the unexpectedly rapid tumor progression during the interval between the two surgical procedures. The literature on brainstem and tumor hematoma in children is reviewed. Received: 26 February 1998     

第三脑室毛细胞型星形细胞瘤的治疗

目的总结第三脑室毛细胞型星形细胞瘤的临床特点和治疗经验。方法回顾分析过去9年间我们收治的8例第三脑室毛细胞型星形细胞瘤的临床资料。本组8例，占同期住院胶质瘤的0．6％，发病年龄平均为19岁，病变在第三脑室前部突向下丘脑／视路的2例，位于第三脑室中后部的6例。8例均行显微手术治疗，肿瘤位于第三脑室前部突向下丘脑／视路的采用经翼点入路；第三脑室中后部肿瘤选择经胼胝体-透明隔入路。结果肿瘤全切6例；2例部分切除，术后给予放射治疗。术后8例患者均治愈出院，7例患者出院随访1年无明显不适。结论毛细胞型星形细胞瘤为良性肿瘤，预后较好，显微手术切除和恰当的术后放射治疗效果较满意。