颅内血管母细胞瘤影像学分型与手术策略的探讨

目的探讨颅内血管母细胞瘤的诊断和治疗。方法回顾性分析58例血管母细胞瘤病人的临床资料,根据MRI检查将肿瘤分为4型：单囊伴单瘤结节型40例,实质型8例,囊实型6例,多发结节型4例。采用后正中、旁正中或枕下人路手术。结果肿瘤全切除56例,大部分切除2例。术后脑肿胀1例,再出血1例,予再次手术。无死亡病例。术后随访1～5年,肿瘤复发2例。结论影像学分型对于指导手术是必要的,应根据瘤结节位置,合理选择手术人路。实质型和囊实型肿瘤手术时间长,手术风险大,术前可行造影检查,必要时栓塞治疗。     

囊性脑膜瘤19例诊治分析

目的 对囊性脑膜瘤的临床特点、影像学表现及其成因进行探讨研究.方法 回顾性分析19例颅内囊性脑膜瘤的临床表现、影像学特征及手术经验.结果 囊性脑膜瘤多位于大脑凸面,多数表现为实性肿块伴大小不等的囊样区,其实质部分明显强化.少数表现为囊性,囊内见实质结节,结节明显强化,而囊壁有或无强化.19例患者肿瘤全切除17例,次全切除2例,无手术死亡.结论 囊性脑膜瘤有特殊的影像学表现, CT和MRI在诊断中具有重要价值.手术应在不损伤神经功能区的前提下全切除肿瘤及囊壁以防止复发.     

颅内血管母细胞瘤的诊断及显微手术治疗

目的 探讨颅内血管母细胞瘤（HB）的诊断方法以及手术治疗。方法 回顾性分析2010年1月~2015年6月手术治疗的54例颅内HB的临床资料。结果 54例中,44例为囊伴结节型肿瘤,8例实质型,2例单纯囊性。肿瘤全切除 52 例,大部分切除2例。术后无再出血,无死亡病例。术后随访1~5年,肿瘤复发4例。结论 MRI是诊断HB的主要方法,影像学分型对于指导手术有帮助,应根据瘤结节位置,合理选择手术入路。     

颅内孤立性纤维瘤2例报告并文献复习

目的探讨颅内孤立性纤维瘤(SFT)的影像学、病理学、临床特征和手术疗效,旨在提高对该病的诊断和鉴别诊断。方法回顾性分析经临床病理证实的2例SFT患者的临床资料,并回顾相关文献。结果2例患者均手术全切除肿瘤,术后随访3年,肿瘤无复发。MRI示肿瘤T1WI多为等信号,T2WI多为混杂信号,增强扫描后不均匀强化。病理免疫组化染色检查:CD34(+),CD99(+),Vim(+)。结论颅内SFT的MRI表现有一定特征性,确诊需依靠病理学及免疫组织化学检查,治疗以手术全切除为主。     

透明细胞型脑膜瘤2例报告并文献复习

目的探讨透明细胞型脑膜瘤的疾病特点、诊断及治疗。方法分析2例罕见的颅内透明细胞型脑膜瘤病例,并复习相关文献。结果 2例肿瘤均位于桥小脑角区,病理组织学特征及免疫组化染色证实为透明细胞型脑膜瘤。肿瘤全切除后均未放疗,随访1a1例复发,1例未复发。结论透明细胞型脑膜瘤好发于椎管内及桥小脑角区,影像学无特征性表现;病理学为良性表现,但有易复发、转移等侵袭性行为,诊断需结合免疫组化;首选手术治疗,对术后肿瘤残留、复发及难以手术的病例,可考虑伽马刀治疗。     

神经内窥镜辅助显微手术切除颅内肿瘤

目的探讨神经内窥镜在辅助颅内肿瘤显微手术中的临床应用价值。方法通过近3年来对颅内听神经瘤、胆脂瘤、大囊小结节型血管网织细胞瘤和较大的脑室内室管膜瘤等15例患者,进行内窥镜辅助显微手术。结果7例听神经瘤患者肿瘤(包括内听道内)全部切除,保存了原有内听道结构,面神经颅内段解剖保留5例(5/7),而内听道段全部保留(7/7)。3例胆脂瘤患者中,2例全切除,1例次全切除。3例大囊小结节型血管网织细胞瘤共6个肿瘤结节,全部切除。2例室管膜瘤中有1例镜下全切除,另1例次全切除。所有15例患者中有13例获得6月至2年随访,疗效满意。结论某些显微镜下视野无法到达的肿瘤病灶部分,在内窥镜辅助下得以安全切除成为可能。     

颅内原发性血管平滑肌瘤诊治及文献复 习简

目的总结颅内血管平滑肌瘤（ALM）的治疗方法及预后。方法回顾性分析2例颅内ALM病例,均行手术切除。复习颅内ALM的相关文献。结果颅内ALM全切除1例,次全切1例。病理学及免疫组化染色结果为具平滑肌细胞的厚壁血管和不规则排列的束带状胶原纤维。病人术后恢复良好,随访至今元肿瘤复发。结论文献报道颅内ALM无影像学特异性。术前均误诊。ALM手术切除预后良好,虽然罕见,术前评估时应考虑到该病的可能性。     

颅内原发性血管平滑肌瘤诊治及文献复习

目的总结颅内血管平滑肌瘤(ALM)的治疗方法及预后。方法回顾性分析2例颅内ALM病例,均行手术切除。复习颅内ALM的相关文献。结果颅内ALM全切除1例,次全切1例。病理学及免疫组化染色结果为具平滑肌细胞的厚壁血管和不规则排列的束带状胶原纤维。病人术后恢复良好,随访至今无肿瘤复发。结论文献报道颅内ALM无影像学特异性,术前均误诊。ALM手术切除预后良好,虽然罕见,术前评估时应考虑到该病的可能性。     

颅内血管母细胞瘤的诊断及显微手术治疗（附34例分析）

且的探讨颅内血管母细胞瘤的诊断和显微手术治疗疗效。方法回顾性分析34例血管母细胞瘤病人的病历资料。肿瘤位于小脑半球27例，脑干区3例，幕上4例。囊性结节样肿瘤31例，实性肿瘤3例。家族性发病3例，诊断为von Hippel-Lindau综合征：复发性2例。均行显微手术治疗，采用神经导航辅助手术10例，锁孔显微手术1例。结果肿瘤全切除30例，次全切除4例。术后重残1例，死亡1例。随访2～10年，2例肿瘤全切除后分别于术后1年、6年复发。结论CT、MRI是诊断血管母细胞瘤的有效检查方法。神经导航对减少术中出血及完整切除瘤结节有较好作用。显微手术是治疗颅内血管母细胞瘤的较佳方案。     

延髓血管网状细胞瘤的显微手术治疗

目的总结延髓血管网状细胞瘤的手术经验。方法回顾性分析48例延髓血管网状细胞瘤的临床资料。肿瘤为囊实型16例,实质型32例。肿瘤单发38例,多发10例。术中采用超声辅助寻找小的瘤结节,在脑干与肿瘤之间的胶质增生带整体切除瘤结节及囊变。结果肿瘤全切除45例,有小的结节遗留3例。随访41例,时间3～74个月,术后症状消失19例,好转18例,加重或出现新的症状4例;复发4例,死亡5例。结论延髓血管网状细胞瘤的显微手术效果良好,关键是在不损伤脑干的前提下,整体切除肿瘤及术后呼吸道的管理。     

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.     

Cystic choroid plexus papilloma with multiple mural nodules in an infant

Background A case of a 13-month-old male infant with progressive increase in head circumference, neurodevelopmental delay, and episodic generalized seizures is reported. He had a history of increase in intracranial pressure because of an arachnoid cyst at the age of 2 months which was managed by cystoperitoneal shunting, but the symptoms were still progressive. Discussion Magnetic resonance imaging revealed an intraventricular cystic tumor with intensely enhancing mural nodules. He underwent surgery and the cyst with the nodules was totally excised. Histopathological findings confirmed the diagnosis of cystic choroid plexus papilloma. Conclusion We recommend precise evaluation of intracranial cystic lesions to prevent wrong diagnosis and operation. Total resection of all enhancing nodules in cystic choroid plexus papilloma is proposed to warrant total cure of tumor.     

Neurocitoma atípico de cerebelo que simula un hemangioblastoma. Presentación de un caso

Through August 2013, 105 cases of intracranial extraventricular neurocytoma (EVN) had been described; 6% were located in cerebellum and 22% were atypical EVN.A rare morphologic form of neurocytoma, atypical EVN has had only 24 cases reported to date. Its prognosis is poorer than the typical central neurocytoma.This case report describes an atypical cerebellar EVN, a form that has not been reported yet, hence the interest of this article. We emphasise its cystic nature and mural nodule, in an infrequent presentation.EVN are low-incidence tumours that we need to take into consideration when making the differential diagnosis of cystic cerebellar lesions with mural nodule.Given that the prognosis of atypical EVNs depends on the atypical nature and on the grade of resection, medical follow up has to be more constant, due to the greater degree of recurrence.     

Unusual CT Hyperattenuating Dermoid Cyst of Cerebellum: A New Case Report and Literature Review

Almost all intracranial dermoid cysts typically display low-density lesions on plain computerized tomography (CT) scans due to abundant lipids content. CT hyperattenuating dermoid cyst (CHADC) is very uncommon with only nine case reports in the literature update, which occurs exclusively in the posterior fossa. Moreover, CHADC with mural nodule is exceptionally rare, and only one such case was documented previously. Here, we report a new case of cerebellar CHADC with mural nodule in a 14-year-old male patient who presented with a 4-week history of dull headache and 5-day history of gait disturbance. With an average attenuation value of 89.9 Hounsfield units on CT scans, the lesion mainly displayed T1 hyperintensity, T2 hypointensity, and FLAIR hypointensity on magnetic resonance imaging. The patient underwent lesion gross total resection and symptomatic improvement, and final pathology was consistent with dermoid cyst. For further clarifying the mechanism of unusual CT hyperdensity, we sampled the cystic content and quantified its protein, calcium, and cholesterol, and our result suggested the high protein, high calcium, and low lipids in contents was the main mechanism of increased CT attenuation for CHADC.     

Tumors of the neuron series presenting as cysts with mural nodules

Two cases of tumors of the neuron series presenting as cysts with mural tumor nodules are described. This radiographic and surgical aspect may be observed in astrocytomas, meningiomas or ependymomas, whereas it is more rare for neuroblastomas and ganglioneuromas. There are not CT features which allow to differentiate cystic tumors of the neuron series from the other cystic tumors. The finding of cyst with enhancing mural tumor nodule and unenhanced wall is more favorable from a surgical point of view; the complete removal of the nodule with preservation of the reactive cyst wall results in the cure of patients with ganglioneuroma and ganglioglioma and in good long-term survival in those with cerebral neuroblastomas.     

脑囊虫病37例临床及影像学特点分析

目的:探讨脑囊虫病的临床及影像学特点,以利于其与颅内其他炎性或肿瘤性病变鉴别。方法:对1995～ 2004年收住我院的37例脑囊虫病的临床及影像学资料,进行回顾性研究。结果:37例脑囊虫病患者,男性25例,女性 12例,年龄6～62岁,平均28岁。经病理证实34例,脑脊液及血清免疫学检查支持3例。临床表现以癫痫样发作为主 者18例(48．6％),以头痛、呕吐、视乳头水肿等颅内压增高为主要表现11例(29．7％),以肢体活动障碍为主者3例(8． 1％),以阵发性眩晕、恶心、呕吐为主者5例(13．5％),头痛伴癫痫发作者4例,头痛伴视力下降者2例,29例无神经系统 阳性体征(80％)。按病变部位并结合影像加以分型:脑实质型27例,脑室型3例,脑膜型5例,混合型2例。结论:单纯 根据临床症候难以确定脑囊虫病,结合MRI观察病灶内是否有头节、病灶囊内密度、囊壁信号等细微变化,同时注意 DWI及增强扫描表现,大多数患者可以得到诊断,少数需应积极进行脑活检。     

Therapeutic results of eight patients with intracranial ependymomas]

Between 1985 and 2001, eight patients with intracranial ependymomas underwent surgery at our hospital. The cases included six infratentorial ependymomas, one supratentorial ependymoma and one supratentorial anaplastic ependymoma. Infratentorial ependymomas were classified according to origin and extension. The lateral type tumors originated from the lateral part of the fourth ventricle in four cases. The midfloor type tumors originated from the inferior half of the fourth ventricular floor in two cases. The three totally resected tumors were the lateral type tumors. The remaining one case with the lateral type tumor underwent nearly total resection of the tumor, since the tumor involved lower cranial nerves. All patients with the midfloor type tumors underwent incomplete resections of the tumors, because the tumors infiltrated into brain stem. Lower cranial nerve involvement and brain stem invasion implied incomplete resection and had the poor prognosis. In intracranial ependymomas, all four patients with total resections have been alive, whereas three of four patients with incomplete resections have died. The mean survival time of all patients with intracranial ependymomas was 127 months from the time of the initial surgery. There were no deaths in the patients with tumors showing MIB-1 index < 10% (n = 4). The mean survival time of the patients with tumors showing MIB-1 index > or = 10% (n = 4), was 30 months. The extent of the resection, the age of the patients and MIB-1 index are important factors in the outcome in patients with intracranial ependymomas. Two representative children aged less than 3 years with the midfloor type tumors were presented. In a patient treated with conventional radiation and chemotherapy, residual tumor repeatedly enlarged within 12 months despite several resections of the tumor. The patient died 32 months after the initial resection. In contrast, the other patient received multidisciplinary treatment including Linac stereotactic radiotherapy (SRT) with a marginal dose of 27 Gy in 9 fractions, have been still alive for 45 months after the initial resection. The residual tumor slightly decreased in size and remained stable without evident growth 12 months after SRT. SRT may provide good local control for patients with intracranial ependymomas and have a favorable impact on survival.     

Pilocytic astrocytoma: unusual feature

Juvenile pilocytic astrocytoma is a well-defined brain tumor. It most often occurs in children and young adults. It is located in the posterior fossa and has typical imaging features, associating cystic and strongly contrast enhancing mural nodule. After complete surgical removal, its prognosis is excellent. Adult cases are seldom observed. They develop almost exclusively within the cerebral hemispheres and share the same imaging and prognostic characteristics as the pediatric forms. We describe the case of a 42-years-old man presenting with a huge heterogenous posterior fossa lesion. Histopathological analysis of the lesion after surgical resection diagnosed a juvenile pilocytic astrocytoma. These peculiar location and imaging features in an adult patient may be misdiagnosed for infectious lesions and must be recognized.     

Pleomorphic Xanthoastrocytoma with an Intracystic Hemorrhage : A Case Report and Literature Review

Pleomorphic xanthoastrocytoma (PXA) has been considered as a low grade tumor of adolescents and young adults. Although this tumor often shows cystic component, the hemorrhage within the cyst is extremely rare. The authors report a rare case of cystic PXA with a hemorrhage within the cyst and the mural nodule in the left frontal lobe. A 64-year-old male presented with a week history of the right side hemiparesis. After gross total resection of the tumor, the patient was fully recovered from neurological deficit. It is suggested that this typically benign tumor could be presented with hemorrhage, causing a rapid neurological deterioration. Prompt surgical intervention, especially total removal of the tumor can provide an excellent functional recovery.