延髓血管母细胞瘤的手术治疗  

Surgical treatment of hemangioblastoma in medulla oblongata：a report of 12 cases

作　　者：严勇[1] 陈菊祥[1] 卢亦成[1] 胡国汉[1] 孙克华[1] 丁学华[1] 骆纯[1] 吴小军[1] 张磊[1] 徐涛[1] 林靖[1] 

机构地区：[1]第二军医大学附属长征医院神经外科上海市神经外科研究所,上海200003

出　　处：《中华医学杂志》2013年第35期2799-2802,共4页National Medical Journal of China

摘　　要：目的探讨延髓血管母细胞瘤（HB）临床特点和诊治策略，提高手术治愈率，减少并发症和死亡率。方法回顾性分析2002至2012年上海长征医院神经外科手术治疗的12例延髓血管母细胞瘤的临床表现、影像学特征、诊治经过和手术效果。结果主要症状为头痛、躯体麻木和肢体肌力下降。磁共振发现病变均为单发。位于延髓近脑桥1例，延髓中部7例，延颈交界4例；位于软膜下9例，髓内3例；3例有囊性变。5例行数字剪影血管造影（DSA），供血动脉主要为小脑后下动脉和小脑前下动脉分支，1例行术前栓塞。完整切除11例，1例仅行电灼。术后症状改善8例，加重2例，死亡2例。随访3个月-10年，McCormick神经功能I-Ⅱ级，1例术后7年复发。结论位于延髓中下段髓内、囊性HB和中小型延髓背侧HB手术安全性较高，术后神经功能可获改善，手术应作为一线治疗选择。延桥交界和位于延髓背侧的大型HB（≥3cm）手术风险大，术后可能出现呼吸衰竭、神经源性肺水肿和脑积水等，手术须慎重。Objective To explore the clinical characteristics, diagnostic strategies and surgical techniques of hemangioblastoma （HB） in medulla oblongata. Methods The clinical and radiological characteristics, therapeutic processes and outcomes of 12 HB cases treated at our department from 2002 to 2012 were studied by retrospective analysis. Results Headache, somatic numbness and limb muscle weakness were the major symptoms of oblongata HB. Magnetic resonance imaging before surgery revealed a total of 12 single tumors. Among these tumors, upper （ n = 1 ）, middle （ n = 7 ） and lower （ n = 4 ） parts of medulla oblongata were involved. The locations were surface （ n = 9 ） and intramedullary （ n = 3 ）. Three tumors had cyst. Digital subtraction angiography （DSA） was performed on 5 cases and it revealed that the main blood supply arteries of tumors were branches of posterior inferior cerebellar artery （PICA） and anterior inferior cerebellar artery （ AICA）. One case underwent pre-surgical embolism during angiography. Eleven tumors were totally resected and 1 was fulgurized. Symptoms improved （ n = 8 ） and worsened （ n = 2）. And two patients died. All survivors were followed up for 3 months to 10 years and had a McCormick functional grading of I- II. One case relapsed 7 year later. Conclusion For Cystic HB, small or medium sized substantial HB in middle and lower part of oblongata, surgical removal is often safe and symptoms may be lessened. It can be used as a first-line treatment. For large （ t〉3 cm） substantial HB or HB in upper part of oblongata, serious postoperative complications such as respiratory failure, neurogenic pulmonary edema or acute obstructive hydrocephalus may occur. Thus surgical resection should be prudently considered and possible consequences thoroughly discussed with the patients.

关 键 词：血管母细胞瘤 脑干肿瘤 显微外科手术 栓塞 

分 类 号：R739.4[医药卫生—肿瘤]