儿童幕上原始神经外胚叶肿瘤的临床病理诊断与显微外科手术治疗

目的分析儿童颅内幕上原始神经外胚层肿瘤的临床、影像学、病理及手术治疗。方法回顾性分析了8例经手术和病理证实的儿童颅内幕上原始神经外胚层肿瘤。结果本组病例肿瘤均位于颅内幕上，影像学上表现有肿瘤呈圆形或类圆形，边界清楚，病灶周围无水肿或水肿较轻；MRIT1WI显示肿瘤实质为等低信号，T2WI为等信号，增强时肿瘤实质明显强化；肿瘤可呈囊性变，部分肿瘤内可见出血。在病理上原始神经外胚层肿瘤由小圆细胞构成，可观察到菊花团形成，免疫组化染色显示肿瘤有多向分化的倾向。本组病例全部进行了显微外科手术治疗，其中4例进行了术后的放射治疗。随访的病例生存期2～16个月，平均8个月。结论需结合临床、影像学和病理特点对原始神经外胚层肿瘤进行诊断，手术后需进行放射治疗。

Clinical, pathological diagnosis and microsurgery of supratentorial primitive neuroectodermal tumors in children

Objective To review the diagnosis and surgical management of supratentorial primitive neuroectodermal tumors in children. Methods The clinical presentation, CT and MRI features and pathological characteristics were analyzed in 8 cases of supratentorial primitive neuroectodermal tumors in children. Results The tumors located in supratentorium were sharply demarcated and there was little edema in the surrounding tissue. The tumor was iso-signal or low signal on T1WI and isosignal on T2WI. The lesion showed marked heterogeneous contrast enhancement on MRL Some tumors contained cysts and hemorrhage. Conventional light microscopy revealed undifferentiated small round cells, and the abortive Homer Wright type rosettes appeared in some cases. Immunohistochemical staining revealed both glial or neuronal markers. Four patients of this group underwent postoperative radiation, and survived for 2 months to 16 months. Conclusions Clinical presentation, CT and MRI pathological features are required for the diagnosis of the primitive neuroectodermal tumors. Post-operative radiotherapy is recommended.