儿童颅内幕上胚胎性肿瘤的治疗及预后分析

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。

Clinical features and prognosis of intracranial embryonal tumors in children

  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 between May 2011 and December 2018 were retrospectively analyzed.  Results  The study included 27 patients, comprising 17 male and 10 female children, with a median age of 7 years. Twelve patients underwent gross total resection, and 15 patients underwent subtotal 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, vtncristine, etoposide, nedaplatin after radiotherapy. The median follow-up period was 22 (4-93) months. Nine patients are alive without local 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 patients with intracranial embryonal tumors. Moreover, the prognosis is poor.