脊髓血管母细胞瘤的MRI诊断与鉴别诊断

MRI diagnosis and differentiation of the spinal cord hemangioblastomas

目的:分析脊髓血管母细胞瘤MRI影像学特征及鉴别诊断。方法:回顾性分析11例脊髓血管母细胞瘤MRI平扫及增强表现,探讨MRI特征,并分析Von Hippel-Lindau综合征(VHL综合征)及非VHL综合征血管母细胞瘤MRI表现的差异。结果:11例中8例为单发肿瘤(72.7%),3例VHL综合征均为多发肿瘤(27个)。MRI表现多与肿瘤大小相关。除体积较小(上下径≤1 cm)者多位于颈髓外,以胸髓多见,邻近背侧软脊膜。肿瘤边界清楚,强化明显。≤1 cm的肿瘤呈点状或结节状,为均匀等T1、稍长T2信号,强化均匀;>1 cm的肿瘤多呈香肠状、葫芦形或不规则形,多以稍长T1、稍短T2为主的混杂信号,不均匀强化。肿瘤常继发大范围脊髓囊性改变(77.8%)及水肿,与肿瘤大小无相关性。>1 cm的肿瘤内部及周围常见流空血管影(100%)。除VHL综合征患者常发生多发、体积小的肿瘤,VHL综合征与非VHL综合征患者在MRI表现中无明显差异。结论:脊髓血管母细胞瘤的MRI影像学表现具有特征性,MR检查是首选方法。

Objective： To analyse the MRI features and differentiation of the spinal cord hemangioblastomas. Methods：The plain and post-contrast MRI of 11 patients with spinal cord hemangioblastomas were analyzed restrospectively. The MRI features and the difference of the MRI features between the patients with VHL syndrome and without VHL syndrome were explored. Results：A total of 27 tumors were found in the 11 patients, 8 patients had solitary tumor（72.7%）, 3 patients with VHL syndrome had multiple tumors. The MR features depended on the size of tumor. Except for small（≤ 1 era） hemangioblastomas which were located in the cervical spinal cord, most hemangioblastomas were located in the thoracic spinal cord, frequently along its posterior aspect. Hemangioblastomas showed well-demarcated,intense enhancement. Small （≤ 1 cm） hemangioblastomas were dotted or nodular shape, showed iso-T1 and slightly long T2 signal intensity and homogeneous enhancement. Large （〉1 cm） hemangioblastomas tended to be sausage, gourd or irregular shape, showed mixed slightly long T1 and slightly short T2 signal intensity and heterogeneous enhancement. Hemangioblastomas were accompanied with extensive spinal code cystic change and edema frequently, which were not correlated with the size of the hemangioblastoma. Large （〉1 cm） tumors invariably had vascular flow voids in or around the tumor （100%）. There was no difference in the MR findings between the two patient groups, except for the multiplicity and higher percentage of small tumors in patients with VHL syndrome. Conclusion： The MR findings of spinal cord hemangioblastomas are characteristic. MRI is the optimal modality.