颅内血管母细胞瘤的MRI表现与临床病理对照

Intracranial hemangioblastoma:MRI findings and pathology correlation

目的探讨血管母细胞瘤的MRI影像表现与临床病理特点,提高其诊断准确率。方法回顾性分析18例经手术病理证实的颅内血管母细胞瘤MRI表现和临床病理特点。结果 18例病灶,5例位于右小脑半球,9例位于左小脑半球,3例位于小脑蚓部,1例位于幕上右侧脑室三角区;MRI表现为大囊小结节型9例,囊实型3例,单纯囊型1例,实质型5例。血管母细胞瘤典型MRI表现为大囊小结节,且小结节显著异常强化。大体病理学检查,肿瘤细胞以囊性和实质性两种形态,囊液透明、淡黄色、蛋白含量高,实性部分由丰富的血管和血窦构成;显微镜下见肿瘤由大小不等的血管腔隙和其间的基质细胞构成,在血管内皮细胞核周围细胞区见丰富的网状纤维;临床上该病好发于中青年,男性为主。结论血管母细胞瘤的MRI表现具有一定特征,结合临床较易诊断;对非典型的血管母细胞,则需进行鉴别诊断,MRI增强更有利于对病变的定性诊断。

Objective The purpose of this study was to analyze the MRI feature and pathological findings of Hemangio- blastomas, so that to elevate the diagnostic accuracy. Methods MR imaging, clinical and pathological features of Heman- gioblastomas in 18 cases were analyzed retrospectively. Results There was a wide distribiadon of lesions. Concretely, le- sions were found in right cerebellar hemisphere （n=5）,left cerebellar hemisphere （n= 9）, cerehellar vermis （n= 3）, and the right lateral ventricle trigone （n=l）.. On MRI, 9 cases were cyst- nodule type, 3 cases were cystic and solid, 1 was pure cystic type, and 5 cases were solid type. The MR imaging was cyst- nodule type of the atypical hemangioblastomas. On contrast-enhanced MR imaging, the nodule component was strongly enhanced, while the wall was enhanced scarcely. 13 were solid and 10 nodules were beside the cyst;typical hemangioblastoma nodules showed large cyst, and small nodules were significantly abnormal enhancement. In general pathology, tumor cells were both cystic and substantive form, cyst fluid and transparent, light yellow, high protein content. The composition of the solid part was the rich in blood vessels and sinusoids. Under the microscope, the composition of tumor cells included blood vessels and the vascular stromal cells, and in the vascular endothelial cel[s around the area, the rich reticular fiber was obserred. It generally occurred in middle- aged male. Conclusion The hemangioblastomas has specific appearances in MRI and can be easily diagnosed combined with clinic, but for the atypical hemangioblastomas, it would take for the differential diagnosis, enhanced MRI is more useful for diagnosing,