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目的探讨Von Hippel-lindau(VHL)病的CT及MRI表现。方法回顾性分析8例临床符合VHL病诊断标准患者的CT和MRI表现以及临床病理资料。8例均行中枢神经系统MRI检查,1例行腹部MRI检查,6例行腹部CT检查。结果 8例均有一处或多处中枢神经系统血管母细胞瘤(2例单发于小脑,6例小脑、延髓及脊髓同时发现病变,1例合并幕上病变)。主要表现为囊实性或实性病灶,增强扫描囊实性病灶呈典型的大囊小结节且结节显著强化,实性病灶增强扫描肿瘤结节明显均匀强化,边界清晰。7例有一处或多处内脏病变,主要表现为胰腺多发囊肿、双肾多发肿瘤和囊肿。结论 VHL病CT及MRI表现具有一定特征性,当出现中枢神经系统多发血管母细胞瘤,胰腺多发囊肿、双肾脏多发肿瘤和囊肿时,则高度提示VHL病的可能性。
Objective To investigate the CT and MRI findings of Von Hippel-lindau (VHL) disease. Methods Retrospective analysis of 8 cases of clinical diagnosis of VHL patients with CT and MRI findings and clinical and pathological data. 8 patients underwent central nervous system MRI examination, 1 case of abdominal MRI examination, 6 cases of abdominal CT examination. Results All of the 8 patients had one or more central nervous system hemangioblastoma (2 lesions were found in the cerebellum, 6 cerebellum, medulla oblongata and spinal cord were found simultaneously, and 1 was associated with supratentorial lesions. The main manifestations of cystic solid or solid lesions, enhanced cystic solid lesions showed a typical large nodules nodules and nodules were significantly enhanced solid lesions enhance the scan of tumor nodules significantly uniform, clear boundary. Seven cases had one or more visceral lesions, mainly for multiple cysts of the pancreas, multiple nephrons and cysts. Conclusions CT and MRI manifestations of VHL have certain characteristics. When central nervous system has multiple hemangioblastoma, multiple cyst of pancreas, multiple tumors and cysts of both kidneys, the possibility of VHL is highly suggestive.