儿童颅内非典型畸胎瘤/横纹肌样瘤预后因素分析

Prognostic factors of intracranial atypical teratoid/rhabdoid tumor in children

目的分析儿童颅内非典型畸胎瘤/横纹肌样瘤(AT/RT)的预后影响因素。方法收集2016年3月至2018年3月期间在首都医科大学附属北京世纪坛医院收治的颅内非典型畸胎瘤/横纹肌样瘤的19例患儿的临床资料。所有患儿均经手术切除肿瘤。其中男13例,女6例。根据患儿确诊时的年龄分为两组(<3岁组和≥3岁组),其中<3岁组7例,先予全身化疗,至患儿年龄>3岁或疾病进展,则予以放射治疗;≥3岁组12例,先予放射治疗,后予化学治疗。结果截至2020年8月30日,19例患儿中有10例患儿存活(52.6%),9例患儿死亡(47.4%)。19例患儿中位生存时间为1.5(0.3-4)年,所有患儿2年PFS和OS分别为(22.8±11)%和(40.7±13.4)%。确诊时年龄及治疗方案是影响AT/RT患儿预后的因素,≥3岁患儿的预后较好,2年OS率为(69.4±15.5)%;手术后联合放疗和化疗的患儿预后较好,2年OS率为(75.8±15.6)%。结论AT/RT患儿总体生存率偏低,尽早采取放射治疗显著提高患儿的生存率。对于小年龄患儿,化学治疗仍是有效的辅助治疗措施。

Objective To analyze the Prognostic factors of intracranial atypical teratoid/rhabdoid tumor(AT/RT)in children.Methods Clinical data of 19 intracranial AT/RT children who were treated in Department of Pediatrics,Beijing Shijitan Hospital,Capital Medical University between Mar 2016 and Mar 2018 were analyzed retrospectively.All patients received tumor resection,including 13 males and 6 females.They were divided into 2 groups according their ages.For the<3 years old group(n=7),all the patients underwent chemotherapy firstly,radiotherapy was not administered until they were 3 years old or disease progression.For the≥3 years old group(n=12),chemotherapy was administered after radiation therapy.Results As of 30th Aug 2020,of the 19 patients,10 patients were alive and 9 dead.The survival rate was 52.6%,while the death rate was 47.4%.The median survival time was 1.5(range 0.3-4)years.The 2-year progression free survival(PFS)rate and 2-year overall survival(OS)rate were(22.8±11)%,and(40.7±13.4)%.The age at diagnosis and treatment strategies were prognostic factors for AT/RT patients.The 2-year OS rate for children≥3 years old was(69.4±15.5)%and it was significantly better than that for patients<3 years old.The prognosis of the patients who underwent surgery combined radiotherapy and chemotherapy was the best and the 2-year OS rate for them was(75.8±15.6)%.Conclusions The OS rate of children with AT/RT is poor.Early radiotherapy could significantly improve the survival rate of patients with AT/RT.For young children,chemotherapy is still an effective adjuvant therapy.