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年轻患者膜软部肉瘤:法国国家NETSARC+网络的经验

Anne-Laure Genevois¹, Matthieu Carton², Myriam Jean-Denis³

¹SIREDO Oncology Centre (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, Paris-Saclay University (PSL), Paris, France.

European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology

|June 19, 2024

概括

膜软部肉瘤 (ASPS) 显示了类似的临床特征,但与儿童相比,年轻人更具侵略性行为. 尽管转移频繁,但两组的长期存活率仍然很高,这表明可比的治疗策略.

科学领域:

儿科瘤学儿科瘤学
肉瘤研究研究肉瘤研究
医学遗传学医学遗传学

背景情况:

膜软部肉瘤 (ASPS) 是一种极其罕见的,耐化疗的肉瘤,主要影响年轻人和儿童.
了解ASPS在儿科与年轻成年人群中的不同临床表现和行为对于优化治疗策略至关重要.

研究的目的:

为了比较小孩和年轻人的膜软部肉瘤 (ASPS) 的临床特征和疾病行为.
评估当前治疗策略的有效性,并确定它们是否应该在儿科和年轻成年人群中标准化.

主要方法:

一项全国性回顾性多中心研究分析了来自ConticaBase肉瘤数据库 (2010-2019) 的数据.
诊断为ASPS的患者被分为儿童 (0-18岁) 和年轻人 (19-30岁).
NETSARC+网络审查了病理学,在所有情况下都确认了TFE3重组.

主要成果:

确定了45名患者:19名儿童和26名年轻人,诊断时临床特征平衡,转移频繁.
治疗主要涉及手术,放射治疗和全身治疗是辅助的. 转移性复发仅发生在最初局部疾病的年轻人中.
五年无事件生存率 (EFS) 在儿童中为74%,在年轻人中为47% (p=0.071),局部瘤的五年无转移生存率 (MFS) 在儿童中为100%,在年轻人中为60% (p=0.005).

结论:

膜软部肉瘤 (ASPS) 呈现出类似的临床特征,但与儿童相比,在年轻成年人中更具侵略性.
尽管在诊断时转移率很高,但儿童和年轻成人患者均表现出有利的长期生存结果.
这些发现支持在儿童和年轻成人患者群体中考虑ASPS的标准化治疗策略.

关键词:

青少年-年轻的成年人膜软部肉瘤是什么意思孩子孩子孩子孩子在NETSARC+上使用.超罕见的肉瘤超罕见的肉瘤

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