目的 探讨脑脊髓血管网状细胞瘤与Von-Hippel-Lindau (VHL)病的MRI影像学特征及诊断与鉴别诊断要点.方法 回顾性分析25例血管网状细胞瘤(其中VHL病1例)的临床表现以及病变在T1WI 、T2WI、T2反转恢复序列、强化MRI扫描序列中的特征.结果 本组肿瘤单发22例,多发3例,共发现肿瘤34枚.分布于小脑28枚,左侧颞叶、脑干各2枚,四脑室内、脊髓各1枚.肿瘤最大径线6.1 cm,最小0.5 cm.大囊小结节型21枚,多为椭圆形或不规则形；实质型12枚,多为圆形或不规则形；单纯囊性型1枚,呈类圆形.1例VHL病,有血管网状细胞瘤5枚,均表现为实质性,同时合并双侧视网膜血管瘤和颈段脊髓空洞.结论 MRI检查对中枢神经血管网状细胞瘤的定位、定性有较高价值.对首发中枢神经血管网状细胞瘤者,应考虑VHL病的可能,及时询问家族史并行相关检查,可早期明确并诊断治疗.%Objective To explore the MRI diagnosis of hemangioblastoma of central nervous system (HCNS) and VHL and the relationship between them. Methods The clinical manifestations and imaging features of Tl WI, T2WI, T2Flair and enhancements of 25 patients with HCNS (among whom 1 was VHL) were retrospectively analyzed. Results Single lesion was found in 22 patients and multiple lesions were found in 3 patients. In total, 34 lesions were found in above-mentioned 25 patients. 28 lesions were on cerebellum, 2 on left frontal lobe, 2 on brain stem, 1 on brain ventricle, and 1 on spinal cord. The largest one was 6.1 cm in diameter and the smallest one was 0.5 cm in diameter. 21 lesions showed large cyst and small nodular and were oval or irregular in shape, 12 were solitary and round or irregular in shape, 1 was simple spina biftda cystica and round in shape. The VHL patient had 5 solitary lesions, accompanied by bilateral retinal hemangioma and cervical spinal cord syringomyelia. Conclusion MRI has important value in its location and qualitative analysis. In patients with HCNS, VHL should be considered. Family history should be inquired and related organs should be examined for the purpose of early diagnosis and treatment.
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