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Surgical management of medulla oblongata hemangioblastomas in one institution: an analysis of 62 cases

机译:某机构的延髓性血管母细胞瘤的手术治疗:62例分析

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摘要

Object: Hemangioblastomas of the central nervous system are highly vascularized benign tumors. When the tumors are located in the medulla oblongata, intraoperative bleeding can make the surgical procedure very difficult. Preoperative embolism has been performed in cases of hemangioblastoma in recent decades. However, the complications of the embolization can result in fatal consequences, especially when the lesions are located in the brainstem. In recent years, selectively blocking the suspicious feeding arteries of the tumors during operation in conjunction with intraoperative neurophysiological monitoring has been performed in the Department of Neurosurgery at the West China Hospital. The purpose of this study is to review all cases that underwent this surgical management and to evaluate their outcomes. Method: Between 2003 and 2014, 62 patients (36 female and 26 male, mean age 35.6 years) underwent microsurgery resection of 67 medulla oblongata hemangioblastomas. The suspicious feeding arteries were identified preoperatively by CTA or DSA. During the operation, the suspicious feeding arteries were blocked selectively by motor evoked potential (MEP) and somatosensory evoked potential monitoring (SEP). Based on the retrospectively review of the clinical records and outpatient long-term follow-up visits, their clinical courses were analyzed. Functional outcomes were evaluated according to the classification of McCormick and the Karnofsky Performance Scale. Result: The maximum tumor diameter ranged from 0.8 to 5.1 cm (mean, 2.9 cm). Total tumor resection was achieved in 60 patients. Sixty-one tumors were removed en bloc, and the other six were resected in a piecemeal fashion. The mean follow-up period was 47 months. During the follow-up period, 34 patients remained neurologically stable, 27 patients recovered to a better status and 16 patients developed new transient neurological dysfunction. One patient died. Karnofsky performance scale scores were 100 in 14 patients (22.9%), 90 in 18 patients (29.5%), 80 in 24 patients (39.3%) and 40 to 70 in 5 patients (8.2%). Seventeen cases were associated with von Hippel-Lindau (VHL) disease. In all the cases, tumor recurrence was observed during follow-up in only 2 patients. Conclusion: This study suggests that safe and effective surgical management of medulla oblongata hemangioblastomas can be achieved for most patients, even without preoperative embolization. With the assistance of intraoperative MEP and SEP, mistaken cutting of the vessels that feed the brainstem can be avoided. With improved microsurgical techniques, intraoperative neurophysiological monitoring and a better understanding of the vascular pattern of tumors, total and en bloc microsurgical removal can be performed with low mortality and favorable prognosis of neurological function.
机译:目的:中枢神经系统血管母细胞瘤是高度血管化的良性肿瘤。当肿瘤位于延髓时,术中出血会使外科手术非常困难。近几十年来,在血管母细胞瘤的病例中已进行了术前栓塞。但是,栓塞的并发症可能导致致命的后果,尤其是当病变位于脑干中时。近年来,在华西医院神经外科,结合术中神经生理学监测,选择性地阻断了手术过程中可疑的肿瘤供血动脉。这项研究的目的是审查所有接受这种外科手术治疗的病例并评估其结果。方法:2003年至2014年间,对62例患者(36例女性和26例男性,平均年龄35.6岁)进行了67例延髓髓性血管母细胞瘤的显微手术切除。术前通过CTA或DSA识别可疑的喂养动脉。手术期间,可动的诱发电位(MEP)和体感诱发电位监测(SEP)选择性地阻塞了可疑的喂养动脉。根据对临床记录和门诊长期随访的回顾性回顾,对他们的临床过程进行了分析。根据麦考密克分类和卡诺夫斯基绩效量表对功能结局进行评估。结果:最大肿瘤直径为0.8到5.1厘米(平均2.9厘米)。 60例患者全部切除了肿瘤。整块切除了61个肿瘤,其余6个被逐个切除。平均随访期为47个月。在随访期间,有34例患者神经功能保持稳定,有27例患者恢复了良好状态,另有16例患者出现了新的短暂性神经功能障碍。一名病人死亡。 Karnofsky绩效量表评分分别为14例患者(22.9%)为100、18例患者(29.5%)为90、24例患者为80(39.3%),5例患者为40至70(8.2%)。十七例与von Hippel-Lindau(VHL)疾病相关。在所有情况下,只有2例患者在随访期间观察到肿瘤复发。结论:这项研究表明,即使没有术前栓塞术,大多数患者也可以安全有效地治疗延髓性血管母细胞瘤。在术中MEP和SEP的帮助下,可以避免错误地切断供入脑干的血管。通过改进的显微外科技术,术中神经生理学监测以及对肿瘤血管模式的更好理解,可以进行整体和整体显微外科切除,而死亡率低且神经功能预后良好。

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