小脑实质性血管网织细胞瘤

目的：探讨小脑实质性血管网织细胞瘤的诊断和治疗。方法：回顾性分析8例小脑实质性血管网织细胞瘤的临床表现及辅助检查，手术方法，结果：术后病人均明确诊断为小脑实质性血管网织细胞瘤，全切难度大。结论：术前应充分考虑到小脑实性变可为实质性血管网织细胞瘤，术前栓塞供血动脉，术中先处理供血动脉和静脉后再切除肿瘤，能降低手术风险和肿瘤复发率。     

颅后窝血管网织细胞瘤及显微外科手术

目的：提高颅后窝血管网织细胞瘤的诊疗水平。方法：回顾总结31例颅后窝血管网织细胞瘤诊断方式及显微外科手术治疗效果。结果：31例肿瘤（囊性23例，实性8例），全切除30例（囊性23例，实性7例），次全切除1例（实性1例），术后神经功能明显改善27例（囊性23例，实性4例），加重3例（均为实性肿瘤），死亡1例（实性）。随访期间有3例复发（囊性1例，实性2例，其中1例实性为颈髓段再发）。结论：明确肿瘤解剖关系及血供情况，囊性肿瘤采用锁孔入路，实性肿瘤骨窗开颅，采用显微外科技术是提高疗效的重要方法。     

第四脑室肿瘤的显微手术治疗

目的探讨第四脑室肿瘤的显微手术方法. 方法 2000年1月～2003年6月我院对29例第四脑室肿瘤采用显微手术治疗,5例因肿瘤侵犯上蚓,瘤体接近皮层部采用小脑蚓部入路,余24例均经小脑延髓裂入路,显微手术切除肿瘤. 结果肿瘤全切除23例,次全切除6例.术后并发症:上消化道出血2例,小脑缄默征1例,第四脑室血肿1例,远隔部位硬膜外血肿1例,呼吸不规则1例.19例随访3～12个月,平均5.5月,肿瘤无复发. 结论术前正确判断肿瘤性质及其基底部所在位置,选择适当的手术入路,熟练掌握第四脑室的显微解剖是手术成功的关键.     

Von Hippel—Lindau病的泌尿系临床和病理观察（附三例报告）

为了提高对Ｖｏｎ Ｈｉｐｐｅｌ－Ｌｉｎｄａｕ（ＶＨＬ）病的认识，对１９９２年１１月到１９９５年１１月收治的３例ＶＨＬ病，就其在泌尿系的临床病理表现、治疗原则及鉴别诊断进行讨论。认为ＶＨＬ病实际是血管壁错构性组织的病变，可遍布身体各器官，其肾实性血管网织细胞瘤极似肾透明细胞癌，但多为良性发展过程。对脑血管网织细胞瘤，一经诊断即应手术切除；对肾实性血管网织细胞瘤，亦应手术切除，但应最大限度保存正常肾组     

颅后窝实体性血管网织细胞瘤显微外科治疗

目的 为了提高颅后实体性血管网织细胞瘤的显微外科治疗效果。方法 回顾总结３４例颅后窝实体性血管网织细胞瘤煌诊断方式及显微外科手术治疗效果。结果 ３４例肿瘤全切除２９例,次全切除５例。手术后神经功能改善２４全理６例,手术后死亡４例,死亡的病例均死于继发性脑干损伤。结论 采用显微外科手术治疗颅后窝实体性血管网织细胞瘤是提高疗效的重要方法。     

小脑囊实性肿瘤诊治分析

目的分析不同年龄段小脑囊实性肿瘤的病理类型、手术切除程度和治疗效果等。方法影像学表现为小脑囊实性肿瘤病人68例,按照年龄分为青少年组36例(年龄18岁)和成年组32例(年龄≥18岁)。根据肿瘤大小和部位选择枕下正中或旁正中入路肿瘤切除术。分析统计两组病人病理类型、手术全切率、并发症发生率和治愈率。结果青少年组以毛细胞星形细胞瘤多见(20例,55.5%),成年组主要是血管母细胞瘤(13例,40.6%),两组比较差异有统计学意义(P0.05)。成年组髓母细胞瘤手术全切率(22.2%)及治疗有效率(33.3%)均低于青少年组(50.0%和44.4%)。两组毛细胞星形细胞瘤及血管母细胞瘤手术全切率均为100%,青少年组毛细胞星形细胞瘤治愈率94.7%,而成年组血管母细胞瘤治愈率为81.8%。两组术后并发症发生率无显著差别。结论小脑囊实性肿瘤在青少年病人中以毛细胞星形细胞瘤多见,成年病人中以血管母细胞瘤为多;两组毛细胞星形细胞瘤及血管母细胞瘤手术全切率高,术后疗效好,而髓母细胞瘤病人手术全切率低,预后较差。     

术前经动脉栓塞脊柱骨巨细胞瘤

目的评价经动脉栓塞治疗脊柱巨细胞瘤（GCT）的临床效果,分析栓塞技术并评价其安全性。方法回顾分析我院2002—2010年共23例（男8例,女15例）脊柱GCT患者,平均年龄29.4岁。栓塞治疗采用PVA颗粒（200～500μm）,2例加用明胶海绵,回顾手术报告评价术中出血。结果 21例患者达到完全栓塞,2例部分栓塞。根髓动脉（RMA）与肿瘤血管共蒂减少了完全栓塞的可能性。23例中9例患者肿瘤同时有RMA供血。完全去血管化患者术中平均出血1950ml,2例部分栓塞患者术中平均出血4800ml,栓塞过程中未出现永久并发症。结论血管造影识别并保护RMA可降低栓塞的风险。术前栓塞脊柱多血管GCT安全、有效,并有助于肿瘤切除。     

颈髓髓内血管网织细胞瘤的诊断及显微外科治疗

目的探讨颈髓髓内血管网织细胞瘤的诊断及显微外科治疗。方法分析26例患者的MRI表现及其显微外科手术治疗的效果。其中男19例，女7例，年龄17～55岁。结果根据肿瘤在MRI上的表现可分为囊肿型12例，实体型14例，均行肿瘤全切除术。术后患者神经系统症状好转者17例，7例无改善，2例加重。结论颈髓MRI能对颈髓髓内血管母细胞瘤作出定位诊断，并可将其分型，以利选择不同的手术方法。颈髓髓内、即使累及延髓的血管母细胞瘤宜行积极手术治疗；根据肿瘤类型不同选择不同手术方法，在手术显微镜下沿正确的界面进行分离，先离断动脉后处理静脉，避免分块切除而力争全切，是减少术中出血和避免神经功能损害的关键。     

手术治疗肌间血管瘤

目的探讨肌间血管瘤的手术方法和临床疗效。方法对7例肌间血管瘤患者,术前行CT、CT血管造影、MRI检查确定血管瘤侵及范围及血供特点,其中3例行手术前介入血管栓塞,1例行颈外动脉临时阻断。结果本组7例患者的疼痛、肿胀症状明显好转,肢体和躯干功能恢复良好。6例经Ⅰ期完整切除后无复发,1例切除后复发经Ⅱ期手术切除。结论早期手术彻底切除瘤体,防止病情快速进展是治疗肌间血管瘤的最有效方法,围手术期对手术难度和并发症要有充分准备;对于瘤体巨大或邻近重要器官的患者,可术前行介入治疗或术中阻断主要供血动脉以控制术中致死性出血的发生。     

颈髓髓内肿瘤显微手术治疗32例

目的 总结颈髓髓内肿瘤显微外科手术治疗的临床经验.方法 回顾性分析应用显微外科手术切除的32例颈髓髓内肿瘤的临床资料.显微镜下分离蛛网膜,于脊髓最薄弱处或后正中沟锐性切开,肿瘤与脊髓之间有可区分界面者,从肿瘤上、下极锐性分离,整块切除肿瘤;肿瘤与脊髓之间无可区分界面者,瘤内分块部分切除病变.结果 32例颈髓髓内肿瘤中,星形细胞瘤18例,肿瘤全切除3例,次全切除11例,部分切除或活检4例;髓内室管膜瘤13例,肿瘤全切除9例,次全切除4例;血管网织细胞瘤1例,仅行减压.术后无并发症发生.结论 应用显微外科手术切除可提高颈髓髓内肿瘤治疗效果和降低并发症的发生.     

Hemangioblastoma in posterior cranial fossa. Report of 56 cases]

The diagnosis of hemangioblastoma in the posterior cranial fossa was confirmed in 56 patients operatively and pathologically. The tumors were cystic in 44 patients and solid in 12; 87.5% of the tumors occurred in the cerebellar hemisphere and 7.1% in the vermis. The main symptoms and signs were increased ICP and cerebellar incoordination. 64 surgical operations had been performed in the 56 patients. Seven patients died and 8 (14%) had recurrence postoperatively. The effect of surgical intervention in 40 patients with cystic hemangioblastoma was satisfactory. Among 5 patients with solid tumor, 2 recovered completely and 3 still had partial neurological disability.     

Attenuation of vascularity by preoperative radiosurgery facilitates total removal of a hypervascular hemangioblastoma at the cerebello-pontine angle: case report

BACKGROUND: The surgical removal of solid, large, and deep-seated hemangioblastomas remains challenging because it is difficult to control bleeding during the procedure. We used preoperative radiosurgery in a solid, highly vascular hemangioblastoma at the left cerebello-pontine angle and present our angiographic, operative, and histologic findings. CASE DESCRIPTION: A 37-year-old paraplegic woman with multiple hemangioblastomas was re-admitted to our clinic with cerebellar ataxia 6 years after resection of a tumor at the fourth ventricle. A vertebral artery angiogram revealed that the 3.5 cm diameter hemangioblastoma at the left cerebello-pontine angle was highly vascular and fed by the left anterior inferior cerebellar artery and posterior inferior cerebellar artery. Nine months before surgical removal it was treated with stereotactic radiosurgery (gamma knife, margin dose 28 Gy) to inhibit tumor progression and to reduce its vascularity. The tumor was totally removed via the left lateral suboccipital approach; bleeding was well controlled and there were no complications. Pathologic examination of the content of the excised tumor revealed coagulation necrosis with hyaline degeneration of the tumor vessels, resulting in a marked decrease in its vascularity. CONCLUSION: Preoperative radiosurgery led to a marked reduction in the vascularity of this hypervascular hemangioblastoma and was useful for controlling bleeding from the tumor during resection. We succeeded to remove the vascular-rich hemangioblastma after the intentional preoperative radiosurgery. The pathologic changes induced by radiotherapy were confirmed by operative finding.     

Delayed diagnosis of cerebellar hemangioblastoma after intracerebellar hemorrhage

BACKGROUND: Cerebellar hemorrhage caused by cerebellar hemangioblastoma is not a frequent case. We report a case of solid, cerebellar hemangioblastoma, diagnosed 4 years after cerebellar hemorrhage. CASE DESCRIPTION: A 69-year-old man presented with dizziness and gait disturbance. He had a 4-year history of evacuation of cerebellar hemorrhage. Gadolinium-enhanced MRI revealed a well-enhancing mass in the left cerebellar hemisphere, and vertebral angiography revealed hypervascularity. Radiotherapy was given to the tumor bed. He remained stable for more than a year after radiation. CONCLUSION: In cases of spontaneous cerebellar hemorrhage, particularly in the patient without hypertension or other underlying diseases related to bleeding from tumor, cerebellar hemangioblastoma should be suspected as a rare cause of hemorrhage, and computed tomography and/or MRI with contrast administration is mandatory for differential diagnosis.     

颅内血管母细胞瘤的MRI表现及病理分析

目的探讨颅内血管母细胞瘤的MRI表现及其病理基础。方法回顾性分析26例颅内血管母细胞瘤的MRI和病理资料,分析其影像学特点。结果单发病灶22例,多发病灶4例,共31个病灶,均位于后颅窝内,其中右侧小脑半球14个,左侧小脑半球9个,4脑室下方小脑下蚓部2个,延髓4个,4脑室2个。19个病灶呈囊结节型、1个病灶呈囊肿型、11个病灶呈实质型或偏实质型。病灶囊性区呈长T1长T2信号,实性区T1WI多呈稍长T1稍长T2信号,其中12个病灶内见稍短T1信号,增强示实性区明显强化,囊性区无强化,3个囊结节型病灶囊壁轻度强化。DWI实性区呈低信号。MRS示胆碱峰明显升高,肌酸及N-乙酰天门冬氨酸峰明显减低或接近消失,可见高尖的脂质及乳酸峰。结论颅内血管母细胞瘤常规MRI及功能成像表现均有一定特征性,MRI对其定位、定性均有较大的临床诊断价值。     

An autopsy case of medulloblastoma with massive hemorrhage

Hemorrhage in medulloblastoma is reported to be extremely rare. This is an autopsy report documenting massive hemorrhage in medulloblastoma in an 11-year-old girl. She had a 3-week history of headache, vomiting and a tendency to fall to the left side, and suddenly developed respiratory arrest followed by coma. At autopsy, there were marked upward herniation of the anterior vermis and left cerebellar hemisphere and downward herniation of the cerebellar tonsils. Horizontal sections through the cerebellum showed a large tumor with a hematoma involving the entire vermis and left part of the cerebellar hemisphere. The histopathological diagnosis was medulloblastoma and no significant changes of tumor vessels were observed. Upward herniation played a significant role in the hemorrhage in this particular case. The presence of upward herniation was not described in the previously reported three autopsy cases of medulloblastoma with hemorrhage.     

Use of gadolinium-enhanced magnetic resonance imaging in the diagnosis and management of posterior fossa hemangioblastomas

The diagnosis of central nervous system hemangioblastoma as well as the surgical treatment requires the accurate radiologic visualization of both the cystic and solid components of the tumor. We report two cases of posterior fossa hemangioblastoma examined with gadolinium-diethylenetriaminepentaacetic acid-enhanced magnetic resonance imaging, which clearly defined the tumor nodule that was not visualized on noncontrast magnetic resonance imaging, contrast-enhanced computed tomography scans, or angiography. In both cases the operative findings precisely correlated with the gadolinium-enhanced magnetic resonance image. Gadolinium-enhanced magnetic resonance imaging is the examination of choice for preoperative evaluation of posterior fossa hemangioblastoma. In cases of von Hippel-Lindau syndrome, magnetic resonance imaging is a useful tool for clinical screening as well as follow-up.     

Glioblastoma multiforme developing separately from the initial lesion 9 years after successful treatment for gliomatosis cerebri: a case report

Gliomatosis cerebri is a diffuse growth pattern of glioma consisting of exceptionally extensive infiltration of at least three cerebral lobes. We report a case of histologically confirmed glioblastoma multiforme in the cerebellar vermis which occurred 9 years after treatment for gliomatosis cerebri. A 33-year-old woman presented to our department for evaluation of visual disturbance. T2-weighted magnetic resonance (MR) imaging revealed hyperintense lesions in the bilateral frontal and parietal lobes. Histological examination of biopsy specimens from the left frontal lobe lesion demonstrated diffuse infiltration of glial neoplastic cells with preservation of the underlying cytoarchitecture, leading to the diagnosis of gliomatosis cerebri. She received 60 Gy hyperfractionated irradiation to the whole brain, and the lesion responded partially. The patient remained stable for 4 years, but T2-weighted MR imaging 5 years after the initial treatment showed enlargement of the hyperintense area. She received nimustine hydrochloride chemotherapy, and again partial response was observed. However. T1-weighted MR imaging after administration of gadolinium-diethylenetriaminepenta-acetic acid detected enhanced lesions in the cerebellar vermis, cerebellar hemisphere, and left posterior limb of the internal capsule 9 years after the initial treatment, although no abnormal findings were observed on initial and follow-up MR imaging. She underwent subtotal removal of the lesion in the cerebellar vermis. The surgical specimens were characterized by dense proliferation of atypical tumor cells with scattered mitosis and endothelial proliferation. The histological diagnosis was glioblastoma multiforme. The patient received gamma knife irradiation for the remnant lesion in the cerebellar vermis, and the lesions in the cerebellar hemisphere and left posterior limb of the internal capsule, and chemotherapy with temozolomide. However, multiple enhanced lesions were detected in the cerebellar vermis 2 months after the start of the temozolomide chemotherapy, and she died 8 months later. This case suggests that glioblastoma multiforme could develop in the long term after initial treatment for gliomatosis cerebri, and in a location separate from the initial lesion.     

Surgical approach to the fourth ventricle cavity through the cerebellomedullary fissure

Lesions of the fourth ventricle represent a challenge to neurosurgeons because of severe deficits that occur following injury to the delicate structures in the ventricle wall and floor. The conventional approach to the fourth ventricle is by splitting the vermis on the suboccipital surface of the cerebellum. In the last 9 years, a series of 21 patients in our clinic underwent microsurgical tumor resection by the unilateral transcerebellomedullary fissure approach. The patients had various pathologies including hemangioblastoma, epidermoid tumor, medulloblastoma, ependymoma, low grade astrocytoma, choroid plexus carcinoma, choroid plexus papilloma, adenocarcinoma in the pons, and cavernoma in the medulla. Total removal was achieved in all but three cases. One death occurred 2 months after surgery due to pulmonary complication. In the follow-up period of 2 months to 5 years, the preoperative symptoms disappeared in all cases except one with a brainstem lesion. By a unilateral transcerebellomedullary fissure approach, it is possible to provide sufficient operative space from aqueduct to obex without splitting the vermis. This approach needs meticulous dissection of the fissure and preservation of the posterior inferior cerebellar artery and its branches.     

Immunohistochemical study is helpful for the diagnosis of cerebellar clear cell ependymoma with atypical radiological findings--case report

A 67-year-old male presented with a clear cell ependymoma with symptoms of ataxic gait and dizziness. Magnetic resonance imaging showed a ring enhanced and circumscribed mass lesion with some cysts in the left cerebellar hemisphere, and the vertebral artery angiogram showed the vascurality of the tumor fed by both the left posterior inferior cerebellar artery and the left superior cerebellar artery mainly. They demonstrated suspicious finding of metastatic tumor, glioblastoma, or cystic meningioma. Surgery via the left suboccipital approach revealed a whitish and solid tumor, which was demarcated from the cerebellar parenchyma and had no continuity with the 4th ventricle. Total resection of the tumor was successfully performed. The hematoxilyn-eosin staining of the surgical specimen was similar to hemangioblastoma or oligodendroglioma, however, immunohistochemical findings for glial fibrillary acidic protein, vimentin, epithelial membrane antigen, and factor VIII were compatible with clear cell ependymoma. The patient's postoperative course was uneventful, and his symptoms improved. Clear cell ependymoma is known as a variant of ependymoma, which is usually located at the foramen of Monro. We think that the immunohistochemical study is highly helpful for the diagnosis of the cerebellar tumor with atypical presentation such as our case.     

Surgical treatment of hemangioblastomas with presurgical endovascular embolization

The usefulness of presurgical embolization of hemangioblastomas was evaluated retrospectively in eight of 30 operations performed on eight of 27 consecutive patients with histologically verified hemangioblastoma. All tumors had over 3.5 cm maximum diameter of the solid mass. Presurgical embolization achieved 100% embolization in one case, 95% to 80% in two, 70% to 50% in two, and less than 50% in three. Total obliteration was obtained only in one case with a single feeding artery. No permanent neurological deficits developed after embolization, but cerebellar infarction occurred in one patient. The surgery was definitely easier than expected in three cases with 80% or more obliteration of tumors. Tumor swelling and cerebellar hematoma occurred during operation in one case with 70% tumor embolization and another case with less than 50%. Blood transfusion during operation was carried out in two cases with less than 50% tumor embolization. The clinical outcome was good recovery in one case, moderate disability in five, and severe disability in two. The reasons for residual neurological deficits were operation and meningitis in one patient, operative and preoperative symptoms in two, and residual preoperative symptoms in four. Neurological deterioration after surgery occurred in three patients with tumor embolization of less than 50%. Partial embolization of hemangioblastomas does not reduce operative complications or morbidity, unless almost complete embolization is achieved, which is not so easy.