中枢神经系统血管母细胞瘤的MRI表现及其病理基础

Hemangioblastomas of the central nervous system:MRI manifestation and pathology

目的:结合病理分析中枢神经系统血管母细胞瘤的MRI影像特点,以提高对其MRI表现的判读水平.方法:搜集我院近3a来经手术病理证实的中枢神经系统血管母细胞瘤21例,所有病例均行MRI常规平扫及增强扫描,对照手术与病理结果分析不同类型血管母细胞瘤MRI的特征表现.结果:病变位于小脑半球及小脑蚓部者共16例,大脑半球1例,椎管内髓内3例,小脑半球、延髓及颈段脊髓多发病灶1例.颅内病变中,12例表现为大囊小结节型;6例表现为实质型;脊髓病变中,2例表现为实质型,1例为大囊小结节型.MRI平扫大囊小结节型囊液与脑脊液信号相似,壁结节呈等信号,实质型病变呈混杂信号,病变内部及周围可见流空血管影;增强扫描壁结节及实性病变显著强化,囊壁及囊液无强化.病理证实壁结节及实性病变由致密的毛细血管网或海绵状血管网组成;囊液由血浆外漏所致,囊壁多由单层内皮细胞组成.结论:不同类型中枢神经系统血管母细胞瘤的MRI影像特点、生长方式与其病理基础相关,明确其病理基础,可进一步认识其MRI影像表现,提高诊断水平.

AIM：To improve diagnosis of hemangioblastomas in the central nervous system （CNS） by means of synthetically analyzing its MRI manifestation characteristics and its pathological basis. METHODS：Twenty-one patients with hemangioblastoma of CNS,confirmed by surgical-pathological findings,were collected from our hospital database over the last three years. And all of them underwent routine and enhanced MRI scan. Combining with the pathological findings,we studied the characteristics of its different types on MRI manifestation. RESULTS ： There were 16 patients whose lesion located in posterior fossa, 3 in spinal cord, 1 in hemisphere, and the last one, presenting multi-lesion, in cerebellum, bulbus medulla and cervical spinal cord. Among the cases with encephalic lesion, 12 present the typical large cyst with small mural nodule, 6 solid mass; among the cases with the lesion in spinal cord, 2 exhibit solid mass and 1 typical large cyst with small mural nodule. On routine MRI findings, the type of heman- gioblastomas with typical large cyst with small mural nodule presents that the signal of its hydatid fluid is similar with that of ncurolymph; the type with small mural nodules presents mediumsignal; the type with solid mass presents mixed signal, otherwise the blood flow signal can be detected inside and around the tumor. On enhanced MRI findings, the type with small mural nodules and the type with solid mass have an obvious enhancement; whereas, the hydatid fluid and the the capsule wall have not any enhancement. It is confirmed by pathology that the small mural nodules and the solid masses are composed of compact capillary network or spongy capillary network, that hydarid fluid origins from transudatory blood plasma, and that capsule wall is composed of monolayer endothelial cell. CONCLUSION： Hemangioblastoma has been divided into 3 types depend on MRI manifestations. Each type＇s characteristic manifestations on MRI and its growth pattern are correlated with the pathological basis. Making clear its pathology can deepen our understanding to its MRI manifestation and improve our diagnosis.