尤因肉瘤(ES)是骨和软组织的恶性肿瘤,最常见于青少年和年轻人。尽管采取了国际协调的方法,几个细微差别,差异,在确定治疗ES的护理标准方面仍存在争议。在这次审查中,作者利用了国家尤因肉瘤肿瘤委员会组建而成的专业知识,一个多机构,多学科虚拟肿瘤委员会每月开会讨论ES的复杂和具有挑战性的病例。本报告的重点是适用于新诊断的ES患者管理的选定主题。涵盖的特定主题包括与氟脱氧葡萄糖-正电子发射断层扫描相比,用于初步评估的骨髓穿刺和活检的适应症,间隔压缩化疗在18岁及以上患者中的作用,转移性疾病患者在长春新碱/多柔比星/环磷酰胺中添加异环磷酰胺/依托泊苷的作用,大剂量化疗自体干细胞移植的数据和作用,维持治疗,和全肺照射。引用的数据通常限于子组分析和/或从多个源编译。虽然不打算取代治疗医生的临床判断,本指南旨在为ES患者的前期管理提供清晰度和建议.尤文肉瘤是一种骨和软组织恶性肿瘤,最常见于青少年和年轻人。对于这篇评论,作者使用了国家尤因肉瘤肿瘤委员会的经验,一个多机构,多学科虚拟肿瘤委员会每月开会讨论尤因肉瘤的复杂和具有挑战性的病例。虽然不打算取代治疗医生的临床判断,该指南的重点是为尤因肉瘤患者的前期治疗制定共识声明.
Ewing sarcoma (ES) is a malignant tumor of bone and soft tissue that most often occurs in adolescents and young adults. Despite an international coordinated approach, several nuances, discrepancies, and debates remain in defining the standard of care for treating ES. In this review, the authors leverage the expertise assembled by formation of the National Ewing Sarcoma Tumor Board, a multi-institution, multidisciplinary virtual tumor board that meets monthly to discuss complicated and challenging cases of ES. This report is focused on select topics that apply to the management of patients with newly diagnosed ES. The specific topics covered include indications for bone marrow aspirate and biopsy for initial evaluation compared with fluorodeoxyglucose-positron emission tomography, the role of interval compressed chemotherapy in patients aged 18 years and older, the role of adding ifosfamide/etoposide to vincristine/doxorubicin/cyclophosphamide for patients with metastatic disease, the data on and role of high-dose chemotherapy with autologous stem cell transplantation, maintenance therapy, and whole-lung irradiation. The data referenced are often limited to subgroup analyses and/or compiled from multiple sources. Although not intended to replace the clinical judgement of treating physicians, the
guidelines are intended to provide clarity and recommendations for the upfront management of patients with ES. PLAIN LANGUAGE SUMMARY: Ewing sarcoma is a malignant tumor of bone and soft tissue that most often occurs in adolescents and young adults. For this review, the authors used the experience of the National Ewing Sarcoma Tumor Board, a multi-institution, multidisciplinary virtual tumor board that meets monthly to discuss complicated and challenging cases of Ewing sarcoma. Although not intended to replace the clinical judgement of treating physicians, the
guidelines will focus on the development of
consensus statements for the upfront management of patients with Ewing sarcoma.