中枢神经细胞瘤7例报告

目的　报道 7例中枢神经细胞瘤 ,探讨中枢神经细胞瘤的临床特点及治疗方法。方法　结合文献回顾性分析 7例颅内中枢神经细胞瘤临床特征。结果　 7例肿瘤均获全切除 ,无特殊并发症 ,无手术死亡。术后均行放疗。结论　中枢神经细胞瘤手术结合放疗为最佳治疗方法 ,预后良好     

中枢神经细胞瘤3例报告

报告3例中枢神经细胞瘤,结合文献探讨中枢神经细胞瘤的病理特点和治疗.     

脊髓髓内星形细胞瘤的临床特点及治疗策略分析

背景与目的：脊髓髓内星形细胞瘤一直是临床治疗的难点，本文旨在分析脊髓髓内星形细胞瘤的临床特点及治疗策略。方法：回顾性分析北京天坛医院2011年5月至2013年5月收治的31例手术治疗的脊髓髓内星形细胞瘤患者的临床资料，包括影像表现、病理类型、手术技巧、术后疗效等，采用McCormick分级和MRI影像学评估手术疗效。结果：肿瘤全切除3例，近全切除15例，部分切除11例，活检2例；术后近期临床神经功能改善1例，无变化23例，加重7例。术后随访3．24个月。20例I～Ⅱ级星形细胞瘤患者中，改善5例，无变化12例，恶化3例；11例高级别星形细胞瘤患者中，好转1例，加重4例，无变化者3例．死亡3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：对边界不清的高度恶性胶质瘤可在激光刀等辅助技术下充分减压后行放化疗等相关治疗．但预后不良。分子靶向治疗可能是今后解决此问题的关键。     

毛细胞型星形细胞瘤的临床特点和外科治疗

背景和目的：毛细胞型星形细胞瘤是一特殊病理类型的星形细胞瘤，本文结合我们的临床病例，探讨毛细胞型星形细胞瘤的临床特点、病理学和影像学特点以及治疗方法。方法：回顾性分析我院近7年来18例经手术病理证实的毛细胞型星形细胞瘤临床资料。结果：18例患者平均发病年龄20岁，大多位于小脑，临床表现主要为颅内压增高症和共济失调，CT和MRI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。病理以镜下见到大量Rosenthal氏纤维为特征性改变。预后与手术切除程度有关，全切组(11例)无肿瘤复发；部分残留组(6例)肿瘤易复发(2例)和恶性变(1例)，未放疗组1例肿瘤复发，为次全切除组。结论：毛细胞型星形细胞瘤好发于年轻人和小脑，有较典型的影像学和病理学特点，应争取外科全切除。对未全切病例术后应行放疗，化疗可作为预防肿瘤复发的一种辅助治疗方法。     

颅内血管外膜细胞瘤的临床诊治6例分析及文献复习

背景与目的：颅内血管外膜细胞瘤（hemangiopericytoma,HPC）发病率低，临床诊治困难，本研究旨在提高对颅内HPC的认识。方法：回顾总结6例颅内HPC患者的临床表现、影像、病理特点以及手术切除和预后情况，并且复习相关文献。结果：6例患者均行开颅手术治疗，其中全切除4例，近全切除2例；术后5例行放射治疗，1例拒绝放疗。术后随访1～10个月，尚未发现肿瘤复发及转移。结论：颅内HPC生长活跃，血供丰富，尤其是位于大脑镰及矢状窦旁时，因此要明确诊断，做好充分的术前准备：手术切除是最佳的治疗手段。应尽量全切，切除范围要广泛．放射治疗是主要的辅助治疗手段。     

颗粒细胞瘤14例临床分析

目的：探讨颗粒细胞瘤的诊断与治疗。方法：1984年-2000年我院采用肿瘤局部扩大范围切除的方法治疗颗粒细胞瘤患者14例，其中男5例，女9例，平均年龄45．2岁。所有病例均经术后病理检查证实。结果：术后随访2。5年，无1例复发。结论：颗粒细胞瘤的诊断依赖于病理检查，手术切除是治疗颗粒细胞瘤的有效途径。     

岛叶胶质瘤的手术治疗

目的：提高岛叶胶质瘤的诊断治疗效果,分析影响预后的因素,提高治疗质量。方法：结合颞叶内侧面的显微解剖特点,回顾性总结近年来我科经外侧裂显微外科治疗的7例岛叶胶质瘤的临床表现、诊断和手术方式的选择与预后及治疗结果。结果：肿瘤全切除4例,次全切除2例,大部分切除1例,术后病理按WHO分级分类,提示星形细胞瘤4例,间变型星形细胞瘤2例,少突胶质细胞瘤1例,术后症状较术前明显改善的有5例,不变1例,加重1例,无手术死亡。结论：利用脑组织的自然解剖间隙,采用显微外科技术切除肿瘤是影响肿瘤切除及其预后的重要因素。     

脑血管母细胞瘤与Von Hippel-Lindau病的诊断与治疗(附1例)

目的：探讨脑血管网状细胞瘤（血管母细胞瘤）与Von Hippel—Lindau病的诊断及治疗，以及两者之间的关系。方法：系统回顾性分析1980年6月-2005年6月诊治的96例脑血管网状细胞瘤，包括其中9例Von Hippel—Lindau病的临床资料。结果：96例脑血管网状细胞瘤全切78例，大部切除18例，死亡6例，手术死亡率6．28％，9例Von Hippel—Lindau病患者中，3例死于肾癌。结论：脑血管网状细胞瘤为良性血管性肿瘤，头颅MR可对典型病例作出定性分析，肿瘤实质性者病程缓慢，囊性者病程较短，出血性者起病急骤。发病部位较为恒定，主要在小脑，而以右小脑半球多见。治疗以手术切除为主，彻底切除则可治愈。Von Hippel—Lindau病具有潜在恶性，有家族遗传倾向。对于首先发现颅内脑血管网状细胞瘤的患者，应考虑Von Hippel—Lindau病的可能，应仔细全身检查，排除其它脏器病变，并长期随诊。     

外侧裂区低级别星形细胞瘤个体化治疗探讨

背景与目的：脑功能区胶质瘤的治疗是神经外科的难题之一。本研究探讨外侧裂区低级别星形细胞瘤的个体化治疗方法，以期达到满意治疗效果。方法：根据术前MRI表现，将56例外侧裂区星形细胞瘤分为4种类型。Ⅰ型：额叶为主型，16例；Ⅱ型：颞叶为主型，13例；Ⅲ型：额-颞跨侧裂型，21例；Ⅳ型：额-颞-岛叶型，6例。依肿瘤特征选择手术进路：Ⅰ型经额前外侧脑沟进路：Ⅱ型经颞前外侧脑沟进路；Ⅲ型和Ⅳ型经外侧裂池进路。在保全神经功能的前提下，显微手术尽可能全切除或多切除肿瘤。术后根据肿瘤级别和特征辅以个体化放疗和，或化疗，平均随访38个月，依据术后MRI和KPS评价治疗结果。结果：肿瘤于镜下全切除39例、次全切除8例、大部分切除6例、部分切除3例：6例遗留永久性轻度神经功能障碍：9例因肿瘤局部复发而行二次手术。术后病理学检查均为WHOⅡ级星形细胞瘤。手术前后KPS无显著性差异。结论：对外侧裂区星形细胞瘤，依据MRI表现，选择个体化的手术进路，应用显微技术，在保全神经功能的前提下，尽可能全切除或多切除肿瘤，最大限度减轻肿瘤负荷，术后辅以个体化放疗和，或化疗，可取得较好的治疗效果。     

脑血管母细胞瘤与Von Hippel—Lindau病的诊断与治疗（附1例）

目的：探讨脑血管网状细胞瘤（血管母细胞瘤）与Von Hippel—Lindau病的诊断及治疗，以及两者之间的关系。方法：系统回顾性分析1980年6月-2005年6月诊治的96例脑血管网状细胞瘤，包括其中9例Von Hippel—Lindau病的临床资料。结果：96例脑血管网状细胞瘤全切78例，大部切除18例，死亡6例，手术死亡率6．28％，9例Von Hippel—Lindau病患者中，3例死于肾癌。结论：脑血管网状细胞瘤为良性血管性肿瘤，头颅MR可对典型病例作出定性分析，肿瘤实质性者病程缓慢，囊性者病程较短，出血性者起病急骤。发病部位较为恒定，主要在小脑，而以右小脑半球多见。治疗以手术切除为主，彻底切除则可治愈。Von Hippel—Lindau病具有潜在恶性，有家族遗传倾向。对于首先发现颅内脑血管网状细胞瘤的患者，应考虑Von Hippel—Lindau病的可能，应仔细全身检查，排除其它脏器病变，并长期随诊。     

中枢神经细胞瘤的M砒和DWI诊断及鉴别诊断

本组收集2002年9月～2011年2月苏州大学附属第一医院收治的8例CNC中,男性4例、女性4例,年龄22～55岁,中位年龄31-3岁。6例患者因头痛、呕吐及视物模糊等颅高压症状入院。     

Benign central neurocytoma

BACKGROUND: "Central neurocytoma" is classically considered as an intraventricular benign tumor, largely based on data from small retrospective series. The authors present prospective data on 12 patients with tumors diagnosed as central neurocytoma, to highlight the diverse nature of this tumor and challenge the classic notion. METHODS: Between 1991 and 1997, 12 patients had tumors diagnosed prospectively as "central neurocytoma". Clinical, radiologic, and histologic data were collected, and Karnofsky performance score was evaluated for each patient. Proliferation marker studies were performed using Ki-67 labeling index. RESULTS: In two patients, the tumors were located in atypical locations, namely, the parietal lobe and the spine. Aggressive behavior characterized by clinical and radiologic evidence of tumor progression was noted in two additional patients. In both these cases, unusually high proliferation rates of 5.3% and 11.2% were noted. Total excision of the tumor, when possible, was the treatment of choice. Postoperative radiotherapy to the residual tumor may be of benefit in patients with clinically aggressive tumors, or those with high proliferation rates. CONCLUSIONS: Given the findings of this study, it is suggested that the traditional concept of central neurocytoma as a benign intraventricular tumor warrants reconsideration.     

Clinical Course of Central Neurocytoma with Malignant Transformation—An Indication for Craniospinal Irradiation

Central neurocytoma is generally considered to be a benign tumor and the literature suggests that a cure may be attained by surgery ± adjuvant focal irradiation. However, there is a need for change in the therapeutic strategy for the subgroup of patients with aggressive central neurocytoma. An example case is presented and the literature on central neurocytoma cases with malignant features and dissemination via the cerebrospinal fluid is reviewed and the radiotherapeutic strategies available for central neurocytoma treatment is discussed. Nineteen cases including the present report with a malignant course and cerebrospinal fluid dissemination have been described to date, most of them involving an elevated MIB-1 labeling index. Our case exhibited atypical central neurocytoma with an initially elevated MIB-1 labeling index (25–30 %). The primary treatment included surgery and focal radiotherapy. Three years later the disease had disseminated throughout the craniospinal axis. A good tumor response and symptom relief were achieved with repeated radiation and temozolomide chemotherapy. Central neurocytoma with an initially high proliferation activity has a high tendency to spread via the cerebrospinal fluid. The chemo- and radiosensitivity of the tumor suggest a more aggressive adjuvant therapy approach. Cases with a potential for malignant transformation should be identified and treated appropriately, including irradiation of the entire neuroaxis and adjuvant chemotherapy may be considered.     

Place de la radiothérapie dans les neurocytomes centraux et revue de la littérature

Central neurocytoma is a rare primary central nervous system tumour of young adults with good prognosis. Typical and atypical forms are described according to various histologic and histopathologic parameters. Central neurocytoma develops in the periventricular areas and is revealed by increased intracranial pressure. The tumour exhibits typical characteristics on CT scan and MRI and a characteristic peak of glycine on spectroscopy-MRI. The main treatment is total resection, which is achievable only in half of the cases. External beam therapy improves local control of partially resected and/or atypical central neurocytoma. Many studies show that stereotactic radiotherapy can be used in the therapeutic management as exclusive treatment, in postoperatives residues and in case of distant recurrence. Chemotherapy is the last line of treatment in refractory forms, especially in the forms with extracranial and/or neuromeningeal spread and in recurrent forms after treatment with surgery and/or radiotherapy.     

Pathologic features and clinical outcome of central neurocytoma: analysis of 15 cases

Objective:To get better recognition of central neurocytoma and diminish misdiagnosis.Methods:A retrospective review identified 15 cases of central neurocytoma.All cases of central neurocytoma were analyzed for their clinical symptoms,pathologic changes,immunohistochemical staining,prognosis and differential diagnosis.Clinical follow up was performed.Results:There were 8 males and 7 females aged 10-64 years(median 32.93 years).The most common presenting symptoms were those related to increased intracranial pressure(ICP),including headache(100%),papilledema(93%) and vomiting(80%).All tumors were located in the ventricular system.The tumors were composed of uniform cells with round nuclei and a fine chromatin pattern,and in some areas,small cells with perinuclear halo could be seen.In particular,the anuclear areas may have a fine fibrillary matrix(neuropil).Nuclear atypia and vascular proliferation appeared in two cases,respectively.Focal necrosis could be seen in one case.Immunohistochemical findings included expression of synaptophysin(15/15),neuron specific enolase(12/15) and glial fibrillary acidic protein(GFAP)(3/15).MIB-1 proliferation index ranged from 0.812.5%,and was more than 2% in 3 of 15 cases assessed.Follow-up information of 11 patients was available.Conclusions:Central neurocytoma has a favorable prognosis in general,but in some cases,the clinical course could be aggressive.Increase of GFAP positivity,proliferation index and vascular proliferation might suggest a more malignant process.     

Coincidence of central neurocytoma and multiple glioblastomas: a rare case report

Coincidence of parenchymal primary brain tumors of different histogenesis is extremely rare. To the best of our knowledge, the present case of simultaneous appearance of a central neurocytoma and multiple glioblastomas is the first to be reported. Multiple intraaxial brain neoplasms were disclosed in a 39-year-old man and were surgically resected. Histological diagnosis of the tumor located in the right lateral ventricle was central neurocytoma whereas two tumors of the left temporal lobe were glioblastomas. The latter were located in close proximity to the subarachnoid space, had atypical radiological appearance, and were slightly positive for synaptophysin and neurofilament protein. It can be speculated that both malignant neoplasms were, in fact, dedifferentiated central neurocytoma, which developed from distant tumor deposits. This case seems to be in agreement with the hypothesis that central neurocytoma arises from the progenitor cells with potential for both neuronal and glial differentiation. Better understanding of histogenesis of this tumor is definitely needed.     

Central neurocytoma: histologic atypia, proliferation potential, and clinical outcome

BACKGROUND: Although central neurocytomas are considered benign, recent reports suggest that some patients with histologic atypia and/or elevated proliferation potential may have a poor outcome. METHODS: A retrospective review identified 15 cases of central neurocytoma. Clinical follow-up was available for 14 patients. Each tumor was evaluated for the presence of atypical histologic features, including cellular pleomorphism, endothelial proliferation, and necrosis. The proliferation potential was assessed by MIB-1 immunohistochemistry. The correlation among histology, MIB-1 labeling index (MIB-1 LI), and clinical outcome was evaluated. RESULTS: Histologic atypia was identified in 3 tumors (20%). The MIB-1 LI ranged from 0.1% to 6.0%, and 5 cases (33%) had an MIB-1 LI >2%. The correlation between histologic atypia and MIB-1 LI was poor, with only 1 tumor having both atypia and MIB-1 LI >2%. Clinical follow-up ranged from 13 to 255 months postoperatively (mean, 68 months). Although most patients were alive and well at last follow-up, three developed symptomatic recurrence and one died as a result of increased tumor growth. The tumors from all 4 patients with a poor outcome had MIB-1 LI >2%, but only 1 had histologic atypia. CONCLUSIONS: The proliferation potential of central neurocytoma is a useful predictor of clinical outcome, whereas histologic atypia alone is not prognostically significant. It would be appropriate to recognize a subgroup of central neurocytomas with elevated proliferation potential as WHO Grade 2 lesions. The terms "atypical" and "anaplastic" are not appropriate to describe these lesions, as they imply a certain histologic appearance. The most accurate designation would be "proliferating neurocytoma."     

Immunocytochemical detection of calcineurin and microtubule-associated protein 2 in central neurocytoma

Summary An immunohistochemical study was carried out on four cases of central neurocytoma, which had characteristic clinicopathological features including ultrastructural findings. Specific antibodies to calcineurin (CaN), microtubule-associated protein 2 (MAP2) and synaptophysin (SYP) were used. All tumor tissues examined showed specific immunoreactivity for CaN and MAP2. Immunolabelling of both molecules revealed that they were mainly localized in the perikarya and proximal processes of the tumor cells. SYP immunoreactivity was found in three of the four cases. SYP immunoreaction products were predominantly seen in the tumor cell processes, while the perikarya were weakly or moderately positive for SYP. The data suggest that CaN and MAP2, together with SYP, can be useful tools for identifying and characterizing of the central neurocytoma.     

Central neurocytoma: Report of 2 cases and literature review

Two cases of central neurocytoma are presented. This is a rare CNS tumour affecting, predominantly, young individuals. There is enough evidence in the literature now to distinguish this entity clinically, radiologically and histologically. Much less is known about the management of patients with this tumour, because most of the reports are in pathological literature.Using our two cases and reviewing the available literature, we are endeavouring to shed light on the clinical aspects and management of central neurocytoma. In particular, we attempted to evaluate the role of surgical excision and radiation therapy in the management of this tumour.     

Central neurocytomas with MIB-1 labeling index over 10% showing rapid tumor growth and dissemination

SummaryObjective and importance Central neurocytoma is recognized as a indolent intraventricular tumor arising from the ependyma around the foramen of Monro and anterior part of the lateral ventricles, and well demarcated from the brain parenchyma. Surgical removal can be curative without postoperative therapy. However, malignant central neurocytoma refractory to even aggressive treatment is known.Clinical presentation We report two cases of extraventricular central neurocytomas with significant vascular proliferation, mitoses, and MIB-1 labeling index of more than 10%.Intervention Subtotal removal for the one patient and open biopsy for other followed by radiotherapy with chemotherapy were performed. However, the disease progressed and dissemination occurred. Both patients subsequently died 23 and 18 month after the histological diagnosis was established.Conclusion Extraventricular central neurocytoma may present with frequent vascular proliferation and high MIB-1 labeling index. Even if they lack malignant histological findings like frequent mitosis and/or necrosis, the prognosis for such patients is very poor.