Objective To investigate the imaging,clinical and pathological features of atypical teratoid/rhabdomyoma(AT/RT)in order to improve the diagnosis and treatment of AT/RT.Methods Retrospective analysis of clinical data of 10 children with AT/RT admitted to the First Affiliated Hospital of Zhengzhou University,and retrieval of domestic and foreign literature for summary and analysis.Results There were 10 cases of AT/RT,including 6 patients under 2 years old(60％),4 patients over 2 years old(40％),4 cases underwent total resection under microscope,and 6 cases underwent subtotal resection.Two patients over 3 years old underwent radiotherapy and chemotherapy.All patients were followed up for 2 to 12 months,and all patients had varying degrees of in situ recurrence during the follow-up period.Among them,1 case had meningeal metastasis and intraspinal metastasis,1 case had concomitant lung metastasis,and 2 cases underwent reoperation due to in situ recurrence and brain metastasis.Conclusion The clinical incidence rate of AT/RT is relatively low.The clinical manifestations are mainly intracranial hypertension.Surgery is usually performed with concurrent chemoradiotherapy.The diagnosis is still dependent on pathological diagnosis.There is no standard chemotherapy and radiotherapy protocol.The tumor is highly malignant and easy to recur,with poor prognosis and high mortality.
关键词
非典型畸胎样/横纹肌样瘤/儿童/临床特征
Key words
atypical teratoid/rhabdoid tumor/child/clinical features
维普
万方数据