颅内血管母细胞瘤的临床分析

目的:探讨不同类型颅内血管母细胞瘤头颅CT、MRI影像特征以及临床特点。方法:采用回顾方法对102例经手术和病理证实的脑血管母细胞瘤进行临床、影像学分析及手术疗效总结。结果:囊腔结节型脑血管母细胞瘤典型表现为囊腔大、结节小及结节显著异常强化;实质性脑血管母细胞瘤信号强度在T2W2明显增高,增强后显著强化。瘤内或瘤周可见血管流空信号。102例手术119次,切瘤126枚,肿瘤全切除114枚,治愈率81.4%,合并VHL术后复发率60.0%(6/10)明显高于散发性5.4%(5/92)。结论:CT和MRI是诊断脑血管母细胞瘤的有效方法,尤其是多轴位成像及增强扫描可避免肿瘤及壁结节漏诊。合并VHL病及多灶病变较散发性脑血管母细胞瘤易复发,易产生新病灶且预后差,应终身随访和定期影像学检查,以便发现新病灶。

Clinical Analysis of Intracranial Hemangioblastoma

Objective:To investigate the proper surgical technique and the CT and MRI characteristic appearances of hemangioblastoma for different types of tumors. Methods:These 102 cases of hemangioblastoma by operation and pathology were analysed retrospectively. The CT and MR imaging characteristic appearances and effects of these operations were summarized.Results:The large cyst and small highly enhanced mural nodule were the characteristic manifestations of the cyst type. Signal intensity of the solid type was very high on T2W2 and solid mass had obvious enhancement. Blood flow phenomena could be found within or around the tumor. These 102 patients were given 119 operations and 126 hemangioblastoma were removed.The total tumor removal was 90.5%(114/126);the satisfactory rate of following-up reached 81.4%(83/102). These patients with VHL disease recurrence accounted for 60.0% of the cases. However,Sporadic cerebellar hemangioblastoma showed recurrence in 5.4%(5/92)of the cases.Conclusions:CT and MRI were the efficient diagnotic methods for hemangioblastomas. MRI can show the local tumors and mural nodules clearly, especially on multi-planar and contrast-enhanced MRI, avoiding missing small lesions. Careful exploration of tumor for cysto-nodular type prevents misdiagnoses.Most of the hemangioblastomas could be removed completely. These patients whose tumors were partially removed received gamma knife radio therapy. These patients with VHL disease and multiple hemangioblastomas had a poor long term prognosis,and tumors tend to recur and new ones occur at risk in contrast to those with sporadic hemangioblastomas. Because patients with VHL disease are at risk for development of new lesions neuroradiological screening allows identification of lesions before they become symptomatic and they require lifelong follow up.