Ewing's sarcoma.

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Abstract

[Indexed for MEDLINE] 5. Cancer. 2024 Dec 1;130(23):4028-4039. doi: 10.1002/cncr.35537. Epub 2024 Aug 25. Consensus recommendations for systemic therapies in the management of relapsed Ewing sarcoma: A report from the National Ewing Sarcoma Tumor Board. Gupta A(1)(2), Dietz MS(3), Riedel RF(4), Dhir A(5), Borinstein SC(6), Isakoff MS(7), Aye JM(8), Rainusso N(9), Armstrong AE(10), DuBois SG(11), Wagner LM(12), Rosenblum JM(13), Cohen-Gogo S(14), Albert CM(15), Zahler S(16), Chugh R(17), Trucco M(16). Author information: (1)Division of Pediatric Oncology, Roswell Park Comprehensive Cancer Center, Buffalo, New York, USA. (2)Department of Pediatrics, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Buffalo, New York, USA. (3)Department of Pediatrics, University of Utah and Primary Children's Hospital, Salt Lake City, Utah, USA. (4)Duke Cancer Institute, Duke University, Durham, North Carolina, USA. (5)Hematology/Oncology Division, Department of Pediatrics, University of Miami Miller School of Medicine, Miami, Florida, USA. (6)Division of Pediatric Hematology/Oncology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA. (7)Center for Cancer and Blood Disorders, Connecticut Children's Medical Center, Hartford, Connecticut, USA. (8)Division of Hematology Oncology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama, USA. (9)Division of Hematology/Oncology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA. (10)Division of Pediatric Hematology/Oncology, Washington University in St Louis, St Louis, Missouri, USA. (11)Dana-Farber/Boston Children's Cancer and Blood Disorders Program and Harvard Medical School, Boston, Massachusetts, USA. (12)Division of Pediatric Hematology/Oncology, Duke University, Durham, North Carolina, USA. (13)Division of Pediatric Hematology, Oncology, and Stem Cell Transplantation, Department of Pediatrics, New York Medical College, Valhalla, New York, USA. (14)Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada. (15)Seattle Children's Hospital/University of Washington School of Medicine, Seattle, Washington, USA. (16)Department of Pediatric Hematology/Oncology/Bone Marrow Transplantation, Cleveland Clinic Children's, Cleveland, Ohio, USA. (17)Division of Hematology/Oncology, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA. 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. © 2024 The Author(s). Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society. DOI: 10.1002/cncr.35537