【摘要】 目的:对儿童颅内幕上胚胎性肿瘤的治疗及预后进行分析,提高对儿童颅内幕上胚胎性肿瘤的认识。方法:回顾性分析首都医科大学附属北京世纪坛医院放疗科2011年5月至2018年12月间收治的27例年龄18岁以下行术后放疗的儿童颅内幕上胚胎性肿瘤的患儿资料,对其临床特点和治疗效果以及预后进行分析。结果:27例患儿均完成术后全脑全脊髓放疗。全脑全脊髓放疗剂量为27.0～30.6 Gy,瘤床区加量照射至55.8～60.0 Gy。22例患儿放疗结束后行化疗,化疗方案为伊立替康+长春新碱+依托泊苷+奈达铂。中位随访时间22(4～93)个月,27例患儿死亡18例,9例生存。1、3、5年生存率分别为79.4%、50.2%、36.5%。死亡患儿中颅内播散8例,脊髓播散10例。结论:儿童颅内幕上胚胎性肿瘤是临床少见的高度恶性神经系统肿瘤,治疗上应采取手术、术后辅助放化疗的综合治疗手段,预后较差。更多还原

【Abstract】 Objective: To investigate the clinical features and prognosis of intracranial embryonal tumors in children. Methods: Clinical data of 27 patients with intracranial embryonal tumors who were admitted to Beijing Shijitan Hospital, Capital Medical University be-tween May 2011 and December 2018 were retrospectively analyzed. Results: The study included 27 patients, comprising 17 male and10 female children, with a median age of 7 years. Twelve patients underwent gross total resection, and 15 patients underwent subto-tal resection. After surgery, all 27 patients underwent craniospinal irradiation. The dose of craniospinal irradiation was 27-30.6 Gy, and the dose of the tumor bed was 55.8-60 Gy. Twenty-two patients underwent chemotherapy regimen composed of irinotecan, vtncris-tine, etoposide, nedaplatin after radiotherapy. The median follow-up period was 22(4-93) months. Nine patients are alive without lo-cal or distant recurrence. Eighteen patients died; the causes of death were intracranial recurrence and spinal cord metastasis. The 1-,3-, and 5-year survival rates were 79.4%, 50.2%, and 36.5%, respectively. Conclusions: Intracranial embryonal tumors in children are rare and malignant and can metastasize along the neuroaxis. Surgery, adjuvant radiotherapy, and chemotherapy are important for pa-tients with intracranial embryonal tumors. Moreover, the prognosis is poor.更多还原