后颅窝、椎管内多发性血管母细胞瘤及von Hippel-Lindau病MRI表现

Multiple hemangioblastoma of posterior cranial fossa and vertebral canal and Hippel-Lindau disease:MRI features

目的观察后颅窝及椎管内多发性血管母细胞瘤的MRI表现。方法回顾性分析经术后病理证实的5例多发性血管母细胞瘤的MRI表现,结合文献分析其影像特征。结果5例血管母细胞瘤患者中2例有明确家族史。MRI共发现32个强化瘤体,其中小脑4个,延髓7个,颈髓10个,胸髓4个,腰髓7个;10个瘤体为单纯结节无囊变,22个伴有囊变,实性瘤体直径范围0.4～1.5 cm,平均直径0.58 cm。结论椎管内血管母细胞瘤MRI表现大多与发生在小脑半球的"大囊小结节"类似,因血供极丰富而强化明显,增强扫描有助于与其他椎管内肿瘤相鉴别。

Objective To observe MRI features of multiple hemangioblastoma of posterior cranial fossa and vertebral canal. Methods MRI features of 5 cases confirmed by his-pathologically were observed and compared to the previous literatures. Results A total of 32 enhanced nodules were found in 5 cases, located in cerebellum （n=4）, bulbus medullae （n=7）, cervical cord （n= 10）, thoracic cord （n=4） or lumbar （n=7）, respectively, and the family history was positive in 2 of 5 cases. Cystic formation was noticed in 22 nodules, whereas the other 10 nodules presented as simple solid tumors. The average diameter of solid nodules was 0.58 cm （0.4-- 1.5 cm）. Conclusion MRI features of hemangioblastorna in vertebral canal are similar to those locating in cerebellar hemisphere. The blood supply of hemangioblastoma is abundant, and thus induces obviously and uniform enhancement on MRI, being helpful to the differential diagnosis of other vertebral canal tumors.