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背景与目的:室管膜瘤与少突胶质瘤混合十分罕见,本文报道我们收治的一例儿童间变性混合型室管膜瘤-少突胶质细胞瘤。方法:回顾性分析一例中枢神经系统间变性混合型室管膜瘤-少突胶质细胞瘤患者的临床资料,综合相关文献报道分析其诊断、治疗和预后。结果:该例患者首次发病年龄为10岁,首发部位为右额叶,在5年内原位复发一次,颅内远隔部位复发3次,先后接受5次手术,3程放疗和多程不同方案的化疗。病理诊断为间变性混合型室管膜瘤-少突胶质细胞瘤。与一般胶质瘤反复在原手术区域局部复发不同,本例患者多次出现颅内新的远隔部位转移病灶,经过反复手术+局部放疗及化疗等综合治疗,病情得到有效控制。结论:病理为间变性混合型室管膜瘤-少突胶质细胞瘤的病例十分罕见,而其生物学特性和治疗预后明显区别于单一成分的间变性室管膜瘤或间变性少突胶质细胞瘤,临床上应予区别对待。
BACKGROUND & OBJECTIVE: The combination of ependymoma and oligodendrocyte is very rare. We report one case of anaplastic mixed ependymoma, oligodendroglioma, in our hospital. Methods: The clinical data of a patient with oligodendroglioma in a patient with central nervous system dysplasia were retrospectively reviewed. The diagnosis, treatment and prognosis of the patients with oligodendroglioma were analyzed retrospectively. Results: The initial age of onset was 10 years. The starting site was right frontal lobe. The site of recurrence was located in situ within 5 years. The recurrence was found in distant sites of the brain. The procedure was followed by 5 procedures, 3 courses of radiotherapy, Chemotherapy. Pathological diagnosis of anaplastic hybrid ependymoma - oligodendroglioma. In contrast to the general recurrence of glioma in the original operation area, the patients showed multiple intracranial new distant metastasis lesions repeatedly. After repeated operation, local radiotherapy and chemotherapy, the disease was effectively controlled. CONCLUSIONS: The pathology of anaplastic hybrid ependymoma, oligodendroglioma, is rare, and its biological characteristics and prognosis are significantly different from those of single component of anaplastic ependymoma or anaplastic oligodendrocyte Stromal cell tumors, should be treated differently.