北京地区婴儿软组织肉瘤的临床特征和预后:一项多中心回顾性研究。

IF 1.4 4区 医学 Q4 ONCOLOGY Journal of cancer research and therapeutics Pub Date : 2023-08-01 DOI:10.4103/jcrt.jcrt_1950_22
Shuai Zhu, Na Xu, Tian Zhi, Yangxu Gao, Dixiao Zhong, Weiling Zhang, Mei Jin, Qing Sun, Yao Xie, Xiaolun Zhang, Long Li, Shengcai Wang, Huanmin Wang, Rong Liu, Weihong Zhao, Dongsheng Huang, Xin Ni, Xiaoli Ma
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引用次数: 0

摘要

背景:婴儿期软组织肉瘤是罕见且研究不足的。在没有这一特殊情况的数据的情况下,我们在中国北京进行了一项基于过去十年收集的多机构队列的婴儿起病肉瘤回顾性研究。我们回顾了婴儿软组织肉瘤的临床特征、治疗方法和结果。材料与方法:对2010年1月至2019年12月在北京市4家初级儿童医院诊断为0 ~ 12月龄的软组织肉瘤患者进行评价。结果:共纳入51例患者,其中男性31例,女性20例。诊断时的中位年龄为5个月(范围0-12),7例(13.7%)患者在出生后第一个月被诊断。组织学上,25例诊断为横纹肌肉瘤(RMS), 6例诊断为骨外尤文氏肉瘤(EES), 20例诊断为非横纹肌肉瘤软组织肉瘤(NRSTS)。RMS的治疗原则和细节主要参考了组间横纹肌肉瘤研究组(IRSG)的治疗方案。对于EES和NRSTS,化疗按照儿童肿瘤组方案进行。RMS的5年EFS/OS率为26.4%±19.5%/56.2±17.8%,EES的5年EFS/OS率为50%±20.4%,NRSTS的5年EFS/OS率为85.2%±9.8%/100%。结论:婴儿期软组织肉瘤具有异质性。RMS和EWS的腹腔或盆腔原发部位较晚,预后较差。多模式治疗成功地控制了大多数患者的疾病。治疗方案的标准化将有助于对这类具有挑战性的疾病进行护理。
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Clinical features and outcomes of infantile soft-tissue sarcoma: A multicenter retrospective study in Beijing.

Background: Soft-tissue sarcomas during infancy are rare and understudied. With no data on this specific condition, we performed a retrospective study of infant-onset sarcomas based on a multi-institutional cohort in Beijing, China, collected over the past decade. We reviewed infantile soft-tissue sarcomas' clinical characteristics, treatments, and outcomes.

Materials and methods: The patients with soft-tissue sarcoma diagnosed from 0 to 12 months in four primary children's hospitals in Beijing from January 2010 to December 2019 were evaluated.

Results: Fifty-one patients were enrolled, including 31 males and 20 females. The median age at the diagnosis was five months (range, 0-12), and seven (13.7%) patients were diagnosed in the first month of their life. Histologically, twenty-five patients were diagnosed with rhabdomyosarcoma (RMS), six were diagnosed with extraosseous Ewing sarcoma (EES), and twenty were diagnosed with nonrhabdomyosarcoma soft-tissue sarcoma (NRSTS). The treatment principles and details of RMS focused on reference to the Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols. For EES and NRSTS, chemotherapy was prescribed according to children's oncology group protocols. The five-year EFS/OS rates of RMS were 26.4% ± 19.5%/56.2 ± 17.8%, the five-year EFS/OS rate of EES was 50% ± 20.4%, and the five-year EFS/OS of NRSTS was 85.2% ± 9.8%/100%.

Conclusions: Infant-onset soft-tissue sarcoma is heterogeneous. The primary location of the abdominal or pelvic cavity of RMS and EWS was at a later stage and had a poorer prognosis. Multimodal therapy resulted in successful disease control for the majority of patients. Standardization of treatment protocols will facilitate care for such challenging conditions.

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来源期刊
CiteScore
1.80
自引率
15.40%
发文量
299
审稿时长
6 months
期刊介绍: The journal will cover technical and clinical studies related to health, ethical and social issues in field of Medical oncology, radiation oncology, medical imaging, radiation protection, non-ionising radiation, radiobiology. Articles with clinical interest and implications will be given preference.
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