摘要
目的探讨脑干血管母细胞瘤(BSHs)的诊断和治疗的进展。方法回顾性分析经外科手术和病理证实、不伴VonHippel-Lindau病的单发BSHs。结果33例BSHs占同期单发颅内血管母细胞瘤的15.5%。男性17例,女性16例,平均年龄45岁。临床表现缺乏特征性,MRI和DSA是主要诊断方法。肿瘤位于延脑14例、桥延脑9例、桥脑6例和延颈髓4例。实质性29例,囊性4例。脑干外型25例,脑干内型8例。小型(≤3cm)5例、大型(3.1～4cm)19例和巨型(>4cm)9例。术前供血动脉栓塞12例。10例亚低温伴/不伴降压麻醉。肿瘤全切31例(94%),不全切2例(6%)。手术死亡2例(6%),均为外生型和实质性肿瘤。31例病人平均随访5年,KPS≥80分25例(80.6%),60～70分4例(12.9%),40～50分2例(6.5%)。结论BSHs多为实质性,可分脑干内型和脑干外型。囊性脑干内型术后疗效优。巨大型实质性BSHs仍然是处理难题。术前供血动脉栓塞,亚低温降压麻醉、显微外科技术和围手术期心肺精心处理等综合措施可提高手术疗效。
Objective To elucidate the advance of diagnosis and surgical treatment of the brainstem hemangioblastomas(BSHs). Methods The data of the following patients treated in one institute were retrospectively analyzed: 1.patients with a single tumor on the brainstem which was verified by surgery and pathology; 2. patients without von Hippel-Lindau disease or multiple hemangioblastomas. Results 33 patients with BSHs were identified, accounting for 15.5% of all intracranial hemangioblastomas surgically treated from Aug 1989 through May 2002 in Shanghai Huashan Hospital. There were 17 males and 16 females. The age of patients ranged from 16 ~ 65 years old with mean of 45 years old. The clinical manifestations were of non-special. MRI and DSA were major diagnostic modalities. Tumors located on oblongata (14), ponto-oblongata (9), pons (6) and cervicomedulla (4). Tumors were solid in 29 cases and cyst in 4 cases, and small size (≤3 cm) in 5, large (3.1 ~ 4 cm)19, and giant (> 4 cm ) 9. Extrabrainstem (EBS) type (including the 4th ventricle hemangioblastomas) was seen in 25 cases, and intrabrainstem (IBS) type was in 8 cases. Preoperative embolization was performed in 12 cases since 1996. Intraoperative mild hypothermia with /without hypotension was done in 10 cases. Total tumor removal was achieved in 31 patients (94%), incomplete removal in 2 cases. Two patients with EBS type and giant solid tumors died after operation. Follow-up study (range from 1 ~ 12 years, mean of 5 years) was available in 31 patients. KPS was ≥80 in 25 patients (80.6%), 60 ~ 70 in 4 patients (12.9%), and 40 ~ 50 in 2 patients (6.5%). Conclusion Two types of BSHs can be identified. Patients with cystic IBS type could obtain excellent outcome after operations. Patients with giant or large solid BSHs are still a challenge to neurosurgeons. A combined strategy of preoperative embolization, mild hypothermia with/without hypotension, microsurgical technique and intensive perioperative management are mandatory for removal of these kind tumors with acceptable morbidity and mortality
出处
《中华神经外科杂志》
CSCD
北大核心
2004年第2期127-131,共5页
Chinese Journal of Neurosurgery
