儿童实质型血管母细胞瘤CT、MRI表现及其病理基础

Solid hemangioblastoma in childhood:CT,MRI findings and their pathological bases

目的分析儿童实质型血管母细胞瘤(solid hemangioblastoma,SHB)的CT和MRI特征及病理特点,提高对本病的诊治水平。方法回顾性分析经病理证实的8例儿童小脑SHB的CT和MRI资料。7例行CT扫描(其中6例行增强扫描),8例均行MRI平扫和增强。结果肿瘤均位于后颅窝小脑区,左侧小脑半球3例,右侧小脑半球2例,第四脑室区3例。影像表现6例SHB为单发圆形或类圆形肿块,边界清楚,2例形态不规则,有分叶;病变CT平扫呈等或稍低密度,MRI平扫T1WI呈等、稍低信号混杂,T2WI呈等、高信号混杂;占位效应明显而水肿轻;增强扫描肿瘤非常显著强化,瘤内及瘤周见多发异常血管影。结论儿童SHB发病率很低,但其CT和MRI表现具有一定的特征性,有助于肿瘤术前定位和定性诊断。

Objective The imaging findings and pathological features of the solid hemangioblastoma （SHB） in childhood were analyzed. Methods CT and MRI findings of eight patients （6 cases of male, 2 cases of female, age range 5 N 14 years old, mean 9. 5 years old） with SHB proved by pathology were analyzed retrospectively. CT plain scan was performed in 7 cases, CT enhancement scan were performed in 6 cases, MRI plain and enhancement scan was performed in 8 cases. Results All patients of SHB located in the posterior fossa （3 cases in the left cerebellar hemisphere, 2 cases in the right cerebellar hemisphere, 3 ca- ses in the region of fourth ventricle）. In 6 patients, the tumor presented as a solitary, oval or rounded and well-defined mass with clear boundary, and in other 2 patients, the tumor was manifested as an irregular mass with lobulated margin. Plain CT showed iso-density and low-density, and plain MRI showed that on TI WI the lesions was characterized by iso-intensity or slightly low-in- tensity signals and on T2WI the lesions exhibited heterogeneous iso-intensity and hyper-intensity signals; the space-occupying effect was obvious with edema; multiple abnormal vessels were observed in tumor and peritumor; MRI enhancement scanning showed the solid tumors were significantly enhanced. Conclusion The incidence of the solid hemangioblastoma in childhood is very low, but CT and MRI performance is of characteristics. CT and MRI examination facilitates the tumor positioning and qualita- tive diagnosis.