Study on the clinical characteristics and prognosis for children high-risk group of head and neck rhabdomyosarcoma

Q4 Medicine

中华实用儿科临床杂志 Pub Date : 2020-03-19 DOI:10.3760/CMA.J.CN101070-20190201-00081

Yi Zhang, Weiling Zhang, D. Huang, Yizhuo Wang, Huimin Hu, Song Li, Xue Meng

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引用次数: 0

Abstract

Objective To study the relationship between high-risk factors with the diagnosis, treatment and prognosis of children with high-risk head and neck rhabdomyosarcoma (RMS). Methods Children with high-risk RMS primarily found in head and neck were selected as research objects according to the criteria of RMS risk degree formulated by Children Oncology Group (COG) and Children′s Rhabdomyosarcoma Cooperative Group, Children′s Hematological Oncology Group, China Anti-Cancer Association (CCCG-RMS), and combined treatment including puncture pathology/surgery, chemotherapy and radiotherapy were performed.The relationship with primary site, age, pathology type and metastasis and prognosis were analyzed. Results Twenty-nine patients were collected as research object, 17 cases (58.6%) of male, and 12 cases (41.4%) of female.The primary site: orbit was 7 patients (24.1%), and para-meningeal was 22 patients (75.9%). Clinical stage: 2 cases were stage Ⅲ (6.9%), and 27 cases were stage Ⅳ (93.1%). Followed up to December 31, 2018, 14 patients were dead, 15 patients were survival, and 10 patients were event free survival.The overall survival rate was 48.3%(15/29 cases), and the event free survival rate was 34.5%(10/29 cases). According to Kaplan-Merier survival curve analysis, the average overall survival time was (76.0±12.0) months, 95% confidence interval (95% CI): (53.5-93.2)months.The event free survival time was (62.5±10.6) months, 95% CI: (47.0-83.9)months. Conclusions The prognosis of high-risk head and neck RMS is poor.The center system metastasis is cause of death.Thus, the main task is how to close follow-up and prevention of central nervous system invasion for improving prognosis. Key words: Rhabdomyosarcoma; Head and neck; High-risk group; Prognosis; Child

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儿童头颈部横纹肌肉瘤高危人群的临床特点及预后研究

目的探讨高危因素与儿童高危头颈部横纹肌肉瘤(RMS)的诊断、治疗及预后的关系。方法根据中国抗癌协会儿童肿瘤学组(COG)和儿童横纹肌肉瘤合作学组、儿童血液肿瘤学组(CCCG-RMS)制定的RMS危险程度标准，选择主要发生于头颈部的高危RMS患儿作为研究对象，进行穿刺病理/手术、化疗、放疗联合治疗。分析其与原发部位、年龄、病理类型、转移及预后的关系。结果共收集29例患者作为研究对象，其中男性17例(58.6%)，女性12例(41.4%)。眼眶7例(24.1%)，脑膜旁22例(75.9%)。临床分期:Ⅲ期2例(6.9%)，Ⅳ期27例(93.1%)。随访至2018年12月31日，死亡14例，生存15例，无事件生存10例。总生存率为48.3%(15/29例)，无事件生存率为34.5%(10/29例)。Kaplan-Merier生存曲线分析，平均总生存时间为(76.0±12.0)个月，95%可信区间(95% CI)为(53.5 ~ 93.2)个月。无事件生存时间为(62.5±10.6)个月，95% CI:(47.0 ~ 83.9)个月。结论高危头颈部RMS预后较差。中心系统转移是死因。因此，如何密切随访，预防中枢神经系统的侵犯，以改善预后是目前的主要课题。关键词:横纹肌肉瘤;头颈;高危人群;预后;孩子

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来源期刊

中华实用儿科临床杂志 Medicine-Pediatrics, Perinatology and Child Health

CiteScore

0.60

自引率

0.00%

发文量

14243

期刊介绍： Chinese Journal of Applied Clinical Pediatrics ( semi-monthly ) is a core journal of paediatrics under the supervision of China Association for Science and Technology, sponsored by Chinese Medical Association and undertaken by Xinxiang Medical College. Founded in 1986, it is openly circulated both at home and abroad. The journal has several columns, such as Expert Forum, Experimental Research and Paediatric Surgery, which are mainly for paediatric medical workers and medical researchers in hospitals. Its purpose is to reflect the new theories and technologies in paediatric medicine and scientific research at home and abroad, and to promote academic exchanges. Chinese Journal of Applied Clinical Pediatrics is a source journal of China Science Citation Database (CSCD), a core journal of Peking University, a source journal of Chinese science and technology paper statistics (China Science and Technology Core Journals), a core academic journal of RCCSE, a high-quality scientific and technical journal of China, a high-quality scientific and technical journal of China Association for Science and Technology, and a high-quality scientific and technical journal of China Biomedical Science and Technology Association. We have been published in China Biomedical Literature Database (SinoMed), China Knowledge Network, Wanfang Data Knowledge Service Platform, China Academic Journal Abstracts, Scopus Database, Chemical Abstracts (USA), Japan Science and Technology Agency (JSTA) Database, Copernicus Abstracts (Poland), Abstracts of the Centre for Agricultural and Biological Sciences (CABS) of the United Kingdom, Cambridge Scientific Abstracts ProQuest Database, WHO Medical Journal of the Western Pacific Region (WMPR), and WHO Medical Journal of the Western Pacific Region (WMPR) of the United States. We have been included in dozens of authoritative databases at home and abroad, such as WHO Western Pacific Region Index of Medicine (WPRIM), Ullrich's Guide to Periodicals, and so on.