AI Summary
The article discusses consensus recommendations for systemic therapies in the management of relapsed Ewing sarcoma, a type of cancer that commonly affects children, adolescents, and young adults. The recommendations are based on discussions by the National Ewing Sarcoma Tumor Board, which focuses on challenging cases of ES. Topics covered include the initial approach to patients with relapsed ES, the role of molecular testing, chemotherapy regimens, novel agents, maintenance therapy, and high-dose chemotherapy with stem cell rescue. The guidelines aim to support clinicians in managing patients with relapsed ES, although they are not intended to replace the clinical judgement of treating physicians.
Abstract
Ewing sarcoma (ES) is a malignant tumor of bone and soft tissue that most often occurs in children, adolescents, and young adults. Debate and controversy remain in the management of relapsed/refractory ES (RR-ES). The authors leveraged the expertise assembled by the National Ewing Sarcoma Tumor Board, a multidisciplinary virtual tumor board that meets monthly to discuss challenging cases of ES. In this review, they focus on select topics that apply to the management of patients with RR-ES. The specific topics covered include the initial approach of such patients and discussion of the goals of care, the role of molecular testing, chemotherapy regimens and novel agents to consider, the role of maintenance therapy, and the use of high-dose chemotherapy with autologous stem cell rescue. 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, these guidelines are intended to support clinicians and provide some clarity and recommendations for the management of patients with RR-ES.
Plain Language Summary
Ewing sarcoma (ES) is a bone and soft tissue cancer that most often occurs in teenagers and young adults.
This article uses the experience of the National Ewing Sarcoma Tumor Board, a multi-institution, multidisciplinary virtual tumor board that meets monthly to discuss challenging cases of ES and to address questions related to the treatment of patients with relapsed ES.
Although not intended to replace the clinical judgement of treating physicians and limited by available data, these consensus recommendations will support clinicians who treat patients with this challenging malignancy, made even more difficult when it recurs.