合并结节性硬化复征的室管膜下巨细胞型星形细胞瘤(附9例报告)

Subependymal giant cell astrocytoma in tuberous sclerosis complex (with report of 9 cases)

目的探讨合并结节性硬化复征的室管膜下巨细胞型星形细胞瘤患者的临床、影像学以及病理学表现特征。方法对1998-2005年收治的9例合并结节性硬化复征的室管膜下巨细胞型星形细胞瘤患者的临床和影像学表现、病理学特点、治疗原则及随访结果进行回顾分析。结果9例室管膜下巨细胞型星形细胞瘤均位于室间孔周围,符合结节性硬化复征的临床诊断标准;男女之比为8∶1。CT检查肿瘤实体部分呈等或稍高密度,肿瘤边界清楚,瘤内有钙化灶。MRI扫描T1WI肿瘤实体部分多呈等信号,少数可呈稍低信号;T2WI呈等或稍高信号;增强扫描肿瘤实体部分呈均匀强化。其中8例同时伴室管膜下结节,5例伴有皮质结节。其中6例经额叶皮质入路,3例经胼胝体-穹窿间入路切除肿瘤;肿瘤完全切除者7例,次全切除(>90%)者2例,无一例手术死亡。随访2～8年,肿瘤完全切除者无复发。结论室管膜下巨细胞型星形细胞瘤为结节性硬化复征的特征性病变,手术完全切除者预后良好。结节性硬化复征所伴室管膜下病变如位于室间孔周围,引起脑积水或呈现逐渐生长趋势者,应考虑室管膜下巨细胞型星形细胞瘤的诊断,尽早施行手术治疗。

Objective To explore the clinical, pathologic and imaging features of subependymal giant cell astrocytoma （SEGA） in tuberous sclerosis complex. Methods From 1998 to 2005, 9 patients with subependymal giant cell astrocytoma and tuberous sclerosis complex were treated in our department. Clinical and imaging presentations, pathologic features, treatment principle, and follow-up findings of these patients were analysed retrospectively. Results All of the tumors located around the foramen of Monro and conformed with the clinical diagnosis criteria of tuberous sclerosis complex. Incidence ratio of male and female was 8 ： 1. In CT scanning, the solid part of tumor showed iso-density or slightly higher density, the tumor border was clear, and calcification was seen in the tumor. On MRI T1WI, the solid part of tumor mostly presented iso-signal while slightly lower signal was lesser and on T2WI iso-signal or slightly higher signal was shown. On enhanced imaging, the solid part of tumor showed uniform intensification. Subependymal tuber was found in 8 cases and cortical tuber in 5 cases. All the tumors were resected. Frontal transcortical approach was performed in 6 cases and transcallosal-interfornix approach in 3 cases. Total resection was achieved in 7 cases, subtotal resection （〉 90%） in 2 cases. No operative death occurred. Followed up 2-8 years. There were no recurrences among the total resected tumors. Conclusion Subependymal giant cell astrocytoma is a characteristic lesion of tuberous sclerosis complex, and it has a good prognosis after total resection. If the subependymal lesion of tuberous sclerosis complex locates around the foramen of Monro and induces hydrocephalus or trends to progressive growing, it should be considered as subependymal giant cell astrocytoma and must be treated earlier.