32例后颅窝血管母细胞瘤的临床特征分析

Clinical Characteristics of Posterior Fossa Hemangioblastoma：a Report of 32 Cases

【目的】探讨后颅窝血管母细胞瘤（HB）的诊断和治疗方法。【方法】回顾性分析自2008年1月至2014年1月本院收治的32例后颅窝 HB患者的临床资料。全部患者行M RI检查，实质性肿瘤4例，囊性肿瘤28例，其中包括3例VHL病（ von Hippel‐Lindau＇s disease）。28例囊性肿瘤行囊结节切除术；4例实质性肿瘤，3例行肿瘤全切除术，1例行次全切除术，残留肿瘤行伽玛刀治疗。【结果】28例囊性肿瘤术后神经系统症状明显改善，4例实质性肿瘤中，3例全切除患者术后症状部分缓解，1例次全切除患者术后因脑积水未解除而行侧脑室－腹腔分流术，术后颅高压症状消失，但肢体麻木无力与术前基本相同。本组患者平均随访时间16个月。囊性肿瘤未发现复发，未遗留严重神经功能障碍，3例V H L病术后6～15个月发现肾细胞癌、肾上腺肿瘤行相关手术治疗。3例行全切除的实质性肿瘤随访10～20个月无复发，但仍有轻度的躯体平衡障碍和后组颅神经症状；行伽玛刀治疗的1例实质性肿瘤，随访7个月，肿瘤无增大，但遗留神经功能障碍，本组无死亡病例。【结论】HB诊断主要依靠M RI检查。治疗首选手术，囊性肿瘤手术效果好；实质性肿瘤特别是位于或邻近脑干时治疗仍较困难，采用综合治疗可提高疗效；VHL病因累及脏器多，易复发，预后较差。

[Objective]To explore the diagnosis and treatment of posterior fossa hemangioblastoma （HB） .[Methods]The clinical data were retrospectively analyzed for 32 HB cases between January 2008 and January 2014 at our hospital .All cases were examined with magnetic resonance imaging （MRI） .Both solid （ n =4） and cystic （ n =28） masses were visualized .And there were 3 cases of von Hippel‐Lindau＆#39;（VHL） disease . All cystic tumors were completely resected .And 4 solid masses were completely （ n=3） and subtotally （ n=2） resected while residual tumor was treated by gamma knife .[Results]Nervous system symptoms of all cystic tumors significantly improved postoperatively .Among 4 solid masses ,the symptom of 3 cases with complete resection achieved partial remission postoperatively .Ventriculo‐peritoneal shunt was performed in one case with subtotal resection due to unresolved hydrocephalus .[Conclusion]The diagnosis of HB is dependent mostly upon M RI examination . Operation remains a first choice and a fair prognosis may be achieved for cystic tumors .The treatment of solid mass especially in brain stem is still challenging .Comprehensive therapy en‐hances the efficacies .The prognosis of VHL disease is still poor due to multi‐organ involvement and frequent recurrence .