近年随着脑肿瘤分子生物学的发展，具有不同组织形态的富含神经毡和真菊形团的胚胎性肿瘤、髓上皮瘤及室管膜母细胞瘤归属于同一分子实体，统称为多层菊花团的胚胎性肿瘤(embryonal tumor with multilayered rosettes，ETMR)。这些肿瘤的罕见性和更新给诊断和治疗带来了挑战。文章主要对ETMR的临床表现、影像特点、诊断及临床研究情况进行回顾分析，以期为临床管理提供指导帮助。对ETMR的临床特点及诊断治疗的研究情况进行文献综述。典型的组织学特点联合分子生物学改变，可以更好地诊断ETMR。目前ETMR的治疗建议主要基于小型、主要是回顾性患者队列的数据。普遍推荐最大安全切除，适应年龄和风险的放疗、化疗为ETMR主要治疗手段，早期放疗可能改善预后。
Advances in the Diagnosis and Treatment of Embryonal Tumor With Multilayered Rosettes in Central Nervous System
In recent years,with the development of molecular biology in brain tumors,Mmbryonic tumors,Ependymoblastoma and Medulloepithelioma with different organizational forms belong to the same molecular entity,which is collectively called the embryonal tumor with multilayered rosettes(ETMR).The rare nature and new changes of these tumors present a challenge for diagnosis and treatment.In this article,we review the clinical characteristics,diagnosis and treatment of ETMR in order to provide guidance and help for clinical management.This article mainly analyzes the clinical manifestations,imaging characteristics,diagnosis,and clinical research of ETMR,in order to provide guidance and assistance for clinical management.A literature review was conducted on the clinical characteristics and diagnostic and therapeutic research of ETMR.The combination of typical histological characteristics and molecular biological changes can better diagnose ETMR.At present,the treatment recommendations for ETMR are mainly based on data from small,mainly retrospective patient cohorts.Maximum safe resection,age appropriate radiotherapy and chemotherapy are the main treatment options for ETMR,and early radiotherapy may improve prognosis.
embryonal tumor with multilayered rosettesdiagnosistreatmentsurgeryradiation therapychemotherapy
万方数据
维普
