儿童颅内伴多层菊形团的胚胎性肿瘤预后相关因素分析:一项单中心回顾性研究  

Analysis of factors associated with the prognosis of embryonal tumor with multilayered rosettes:a single-center retrospective study

作　　者：郁佳华 王成[2] 余颖 王雅菲 张晨冉 YU Jiahua;WANG Cheng;YU Ying;WANG Yafei;ZHANG Chenran(Department of Pediatric Neurosurgery,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China;Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China)

机构地区：[1]上海交通大学医学院附属新华医院儿神经外科,上海200092 [2]上海交通大学医学院,上海200025

出　　处：《临床儿科杂志》2025年第4期293-300,共8页Journal of Clinical Pediatrics

基　　金：国家自然科学基金面上项目(No.82170798)。

摘　　要：目的回顾性研究影响儿童颅内伴多层菊形团的胚胎性肿瘤(ETMR)预后的主要因素,为改善患儿的预后提供有效的建议。方法收集2013年1月至2023年12月共11年在手术后确诊的ETMR患儿29例。数据来源于住院病历及电话随访内容。最后接受随访的17例患儿被纳入分析。回顾性分析17例ETMR患儿的临床资料,总结患儿的性别、年龄、肿瘤位置、临床表现、手术切除程度、术后放化疗、术后转移和复发的情况及预后。结果其中11例患儿诊断为ETMR,2例诊断为具有丰富神经纤维和真菊形团的胚胎性肿瘤(ETANTR),4例病理为髓上皮瘤(MEPL),确诊时的中位年龄为2.5岁(11个月20天到8.6岁)。研究终点为患儿死亡,中位生存时间为8个月(0至130.5个月)。患儿的1年无进展生存(PFS)率为18%,1年总生存(OS)率为41%,3年PFS率为18%,3年OS率为24%。死亡13例(76%),死亡原因为肿瘤引起的相关并发症、肿瘤进展或复发。生存分析结果中,性别、术后行放疗、术后行化疗、术后行联合放化疗对于总体生存时间的影响有统计学差异(P<0.05)。结论ETMR为高度侵袭性,病死率高,其治疗通常从最大限度的手术切除开始,手术全切、术后行放疗及化疗与较好的预后相关。Objective The purpose of this study was to retrospectively analyze the main factors affecting the prognosis of pediatric intracranial embryonal tumor with multilayered rosettes(ETMR)and to provide effective suggestions for improving the prognosis of children with this condition.Methods From January 2013 to December 2023,clinical data were collected from 29 patients with ETMR who underwent surgery and were diagnosed postoperatively.Data were obtained from medical records and telephone follow-ups.A total of 17 patients who completed follow-up were included in the analysis.The clinical data of these 17 children with ETMR were retrospectively reviewed,summarizing their gender,age,tumor location,clinical manifestations,extent of surgical resection,postoperative radiotherapy and chemotherapy,recurrence and metastasis,as well as prognosis.Results Among the 17 patients,11 were diagnosed with ETMR,2 with embryonal tumor with abundant neuropil and true rosettes(ETANTR),and 4 with medulloepithelioma(MEPL).The median age at diagnosis was 2.5 years(range:11 months and 20 days to 8.6 years).The study endpoint was death,with a median survival time of 8 months(range:0 to 130.5 months).The 1-year progression-free survival(PFS)and overall survival(OS)rates were 18%and 41%,respectively,while the 3-year PFS and OS rates were 18%and 24%,respectively.Thirteen patients(76%)died due to tumor-related complications,progression,or recurrence.Survival analysis showed that gender,postoperative radiotherapy,chemotherapy,and combined chemoradiotherapy had statistically significant effects on overall survival(P<0.05).Conclusion ETMR is a highly invasive tumor with a high mortality rate.Treatment typically begins with maximum surgical resection.Gross total resection,postoperative radiotherapy,chemotherapy,or combined chemoradiotherapy are associated with better prognosis.

关 键 词：颅内伴多层菊形团的胚胎性肿瘤 手术 放疗 化疗 儿童 

分 类 号：R739.41[医药卫生—肿瘤]