小脑毛细胞型星形细胞瘤的诊治分析

目的探讨小脑毛细胞型星形细胞瘤的临床诊断及显微手术治疗效果。方法对经病理证实的17例小脑毛细胞型星形细胞瘤的影像学表现、组织病理学特征、手术治疗方法及预后等进行分析、总结。结果小脑毛细胞型星形细胞瘤以20岁以下发病率最高;影像学上肿瘤可分为3种类型:完全囊肿型(2例,12%),囊肿结节型(7例53%),肿块型(6例35%)。病理学镜下表现为双极毛发样或纤维状细胞,伴Rosenthal纤维。手术所见肿瘤与周围组织间有相对清楚的边界。肿瘤全切除15例,大部切除2例。结论小脑毛细胞型星形细胞瘤有比较典型的影像学和病理学特征,显微手术全切是首选治疗方法,预后良好。     

黏液性毛细胞型星形细胞瘤

黏液性毛细胞型星形细胞瘤是一种最近才被描述的中枢神经系统肿瘤,其特点类似于毛细胞型星形细胞瘤,过去被归为毛细胞型星形细胞瘤的一个亚型。然而,最近的研究表明,黏液性毛细胞型星形细胞瘤的组织学特点和毛细胞型星形细胞瘤有细微的差异,在生物学特性上,两者之间在侵袭性,复发率和预后都有不同。提示了黏液性毛细胞型星形细胞瘤是一类不同于毛细胞型星形细胞瘤的肿瘤。深入了解毛细胞型星形细胞瘤和黏液性毛细胞型星形细胞瘤的组织学特点,临床表现,影像学特点,治疗原则,预后对进一步开展相关研究有重要意义。     

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。     

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。     

罕见钙化性毛细胞型星形细胞瘤一例

患者　男 ,10岁。以轻度反复发作前额头疼 5年。间断性右面及上肢抽搐、伴行走不稳 3年。近 1个月症状加重并呕吐入院。查体 :神清合作 ,瞳孔左∶右 =3∶3ｍｍ ( ) ,双视乳头早期水肿、眼震 (- )、角膜反射 ( )、咽反射对称灵敏、蹒跚步态、右侧肢体共济差、双巴彬斯基征(± )。头颅平片后颅窝团块状高密度钙化影像。头颅ＣＴ平扫右小脑半球及蚓部不规则斑块状高密度钙化影 ,其间低密度 ,周围无水肿 ,第四脑室受压、移位变形、幕上脑室扩大 ;增强扫描病灶无强化 ,印象结核瘤。头颅ＭＲＩ显示同样部位Ｔ1、Ｔ2均呈高、低、等混杂信号 ,形态不…     

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。     

脊髓毛细胞型星形细胞瘤5例临床分析

目的 探讨脊髓毛细胞型星形细胞瘤(PA)的临床、影像学、病理学特点及治疗与预后.方法 回顾性分析5例脊髓PA患者的临床资料.5例患者均行手术全切除肿瘤,术后随访观察6～36个月.结果 本组5例患者均为成年人;术前MRI检查示肿瘤位于脊髓颈段2例、胸段1例、腰段1例、颈至腰段1例;首发症状为肢体疼痛4例、肢体麻木2例、肢...     

脑毛细胞型星形细胞瘤的MRI诊断特点(附32例报告)

目的探讨脑毛细胞型星形细胞瘤的MRI表现,以提高诊断正确率。方法回顾性分析32例经手术病理证实的毛细胞型星形细胞瘤的临床及MRI特点。结果32例中,71.9%发生于20岁以下青少年人,其中小脑蚓部10例(31.3%),小脑半球9例(28.1%),鞍区7例(21.9%),基底节区和脑干各2例(6.3%),第三脑室内1例(3.1%),右侧颞叶1例(3.1%)。MRI上分四型:单囊型14例(43.8%)、多囊型12例(37.5%);实性型5例(15.6%);完全囊变型1例(3.1%)。肿瘤实性部分平扫T1WI为等或稍低信号,T2WI为等或稍高信号;囊性部分T2WI信号多比脑脊液信号高。增强扫描实性部分明显强化,呈蜂窝状。灶周无水肿28例(87.5%);4例灶周轻微水肿。结论毛细胞型星形细胞瘤的MRI表现有一定的特征性,结合患者的年龄及临床表现可以提高术前诊断率。     

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。     

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.     

Anaplastic pilocytic astrocytoma

This clinical series examines the presentation of three adult patients who were found to have de novo anaplastic pilocytic astrocytoma. Initial imaging demonstrated an intracranial mass with histological analysis diagnostic of pilocytic astrocytoma with anaplastic features including necrosis, marked nuclear pleomorphism and a very high mitotic rate leading to the diagnosis of anaplastic pilocytic astrocytoma. We discuss the clinical pitfalls, treatment and implications when managing this condition.     

颅内毛细胞型星形细胞瘤的诊治及预后

目的探讨颅内毛细胞型星形细胞瘤的病理学特点、临床特点、影像学表现和治疗经验。方法回顾性分析南京军区总医院神经外科2002年6月至2006年6月四年间20例经手术病理证实的毛细胞型星形细胞瘤临床资料。分析其影像学特点及术中所见和预后情况。结果20例肿瘤均接受显微外科手术,根据肿瘤所在部位采取不同手术入路。16例全切,4例大部分切除,4例均位于视交叉或下丘脑部,与周边重要神经结构粘连较紧。结论颅内毛细胞型星形细胞瘤为良性肿瘤,显微手术全切预后良好,对于未全切病例术后行恰当的放疗或化疗也能获得理想效果。     

Long-term functional outcome of surgical treatment of juvenile pilocytic astrocytoma of the cerebellum in children

Purpose  Increasing incidence of pediatric brain tumors and improving survival rates encouraged us to assess long-term functional outcome of patients with cerebellar juvenile pilocytic astrocytoma (JPA). Materials and methods  Our study encompassed 105 children treated since 1980–2005 and consisted in analysis of mailed, custom-designed questionnaires. Results  Mean follow-up time was 8.3 years. Sixty out of 104 patients presented permanent neurological deficits and 47/104 presented significant behavioral disorders. Eighty-nine children continued their education at primary, secondary or high school level. Most patients and their parents were satisfied with treatment outcome. Patients’ and parents’ notes were usually concordant. Conclusions  Long-term functional treatment outcome of cerebellar JPA is relatively favorable, in spite of permanent neurological deficits and emotional disorders in over half of the patients. Vermian tumors are associated with worse long-term functional outcome. Neurological deficits and emotional disorders do not preclude further education and independent functioning.     

Diversity of glial cell components in pilocytic astrocytoma

To characterize the cellular density and proliferative activity of GFAP‐negative cells in pilocytic astrocytoma (PA), surgically excised tissues of PAs (n = 37) and diffuse astrocytomas (DAs) (n = 11) were examined morphologically and immunohistochemically using antibodies against GFAP, Olig2, Iba1 and Ki‐67 (MIB‐1). In PA, Olig2 immunoreactivity was significantly expressed in protoplasmic astrocytes in microcystic, loose areas and cells in oligodendroglioma‐like areas. Iba1‐positive, activated microglia/macrophages were also commonly observed in microcystic areas. In compact areas, a prominent reaction for GFAP was observed, but for Olig2 and Iba1 to a lesser degree. On semiquantitative analysis, the number of Olig2‐positive cells was significantly higher in PAs (mean labeling index (LI) ± standard deviation (SD): 46.8 ± 15.4%) than in DAs (13.3 ± 7.8%) (P < 0.001). Many Iba1‐positive, microglia/macrophages were observed in PAs (19.9 ± 6.5%), similarly to DAs (20.9 ± 9.9%). Re‐immunostaining of PA demonstrated that most Ki‐67‐positive, proliferating cells expressed Olig2, whereas GFAP or Iba1 expression in Ki‐67‐positive cells was less frequent (14.7 ± 13.7%, and 8.8 ± 13.6%) in a double immunostaining study. Conversely, the percentage of Olig2‐positive, proliferating cells in total Olig2‐positive cells (7.2 ± 3.9%) was higher than that of Iba1‐positive, proliferating cells in total Iba1‐positive cells (0.9 ± 0.6%). In conclusion, the present study found that PA consisted of numerous GFAP‐negative cells, including Olig2‐positive cells with high proliferation. Semiquantitative analysis of Olig2 immunohistochemistry in microcystic areas might therefore be useful for the differential diagnosis of PA and DA.     

Congenital anaplastic astrocytoma differentiated into pilocytic astrocytoma: An autopsy case

We report an autopsy case of congenital astrocytoma and its histopathological changes during 5 years of the patient's development from birth to death. At birth, a right exophthalmic tumor was observed, and MRI revealed that the tumor occupied the right orbital space and had also affected the suprasellar diencephalic structures. The right orbital tumor, which was enucleated at 2 months of age, was a highly cellular tumor with moderate pleomorphism resembling anaplastic astrocytoma. On the other hand, at autopsy, a brain tumor was found in the right diencephalic region with features of pilocytic astrocytoma, accompanied by leptomeningeal dissemination. A biopsy specimen, which was obtained from the chiasmatic part of the tumor at 4 months of age, showed an intermediate appearance between the orbital tumor and the brain tumor obtained at autopsy. Immunohistochemical examination confirmed that all three phases of the tumors showed an astrocytic lineage, and the Ki‐67 labeling index decreased rapidly after 2 months of age. We believe that this congenital anaplastic astrocytoma differentiated into a pilocytic astrocytoma during the 5 years of the patient's development. The transformation of the congenital astrocytoma from anaplastic to pilocytic forms can be attributed to the nature of the tumor, namely postmitotic neoplastic cells are characterized by their ability to undergo self‐differentiation, along with the organotropism of the developing brain.     

儿童毛细胞型星形细胞瘤术后综合治疗研究进展

毛细胞型星形细胞瘤（PA）是最常见的儿童低级别脑肿瘤,常见于视神经、视交叉、下丘脑、脑干、小脑和大脑等部位。PA往往边界清楚、生长缓慢,属于WHO I级肿瘤,手术切除是治疗的首选方案。然而,由于视神经、视交叉、下丘脑或脑干等处神经功能的重要性,手术不一定能做到全切。目前临床对于儿童PA术后的治疗方案尚无定论,国内外对于儿童PA术后的主要治疗方式包括放疗、化疗、靶向治疗和随访观察。随着影像学和放射技术的进步,除了传统分次放疗,还包括适形放疗、立体定向放射治疗和质子治疗。但目前多数学者尚不推荐将放疗作为儿童PA术后的一线治疗选择。以长春新碱联合卡铂的化疗方案是进展性PA的首选,伊立替康-贝伐单抗可作为二线治疗用药。靶向治疗的发展如火如荼,以丝裂原活化蛋白激酶（MEK）抑制剂为首的靶向药物因其更少的毒副反应和良好的疗效而有望在未来成为儿童PA术后的一线用药,但目前尚缺乏高级别的临床证据。随访观察是儿童PA术后的选择之一,有学者提出儿童PA的分层治疗方案,推荐低风险层患者于术后随访观察。该文对儿童PA术后综合治疗进展作综述报道。