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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。
Objective To analyze the clinical, radiological, pathological and surgical treatment of primary neuroectodermal tumors in children with intracranial supraoptic tumors. Methods A retrospective analysis of 8 cases of surgically and pathologically confirmed children’s intracranial supraoptic primitive neuroectodermal tumors. Results The tumors in this group of patients were all located in the intracranial supraspinal surface. The imaging showed that the tumors were round or round, and the border was clear with no edema or edema around the lesion. MRIT1WI showed that the tumor parenchyma was low signal and T2WI was equal signal , When the tumor was enhanced significantly enhanced; cystic tumor can be changed, some tumors showed bleeding. Pathologically, primitive neuroectodermal tumors were composed of small round cells, and chrysanthemum formation was observed. Immunohistochemical staining showed the tendency of the tumors to differentiate into multiple directions. All patients in this group were treated with microsurgery, of which 4 patients underwent postoperative radiotherapy. Survival of follow-up cases 2 to 16 months, an average of 8 months. Conclusion The primary neuroectodermal tumors need to be diagnosed in combination with clinical, radiological and pathological features. Radiotherapy should be performed after the operation.