Consensus recommendations in the management of Ewing sarcoma from the National Ewing Sarcoma Tumor Board

Ajay Gupta; Richard F Riedel; Chirag Shah; Scott C Borinstein; Michael S Isakoff; Rashmi Chugh; Jeremy M Rosenblum; Erin S Murphy; Shauna R Campbell; Catherine M Albert; Stacey Zahler; Stefanie M Thomas; Matteo Trucco

doi:10.1002/cncr.34942

Consensus recommendations in the management of Ewing sarcoma from the National Ewing Sarcoma Tumor Board

Cancer. 2023 Nov 1;129(21):3363-3371. doi: 10.1002/cncr.34942. Epub 2023 Jul 5.

Authors

Affiliations

¹ Division of Pediatric Oncology, Department of Pediatrics, Roswell Park Comprehensive Cancer Center, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Buffalo, New York, USA.
² Duke Cancer Institute, Duke University Medical Center, Durham, North Carolina, USA.
³ Department of Radiation Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio, USA.
⁴ Division of Pediatric Hematology-Oncology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
⁵ Center for Cancer and Blood Disorders, Connecticut Children's Medical Center, Hartford, Connecticut, USA.
⁶ Department of Medicine, Division of Hematology and Oncology, Rogel Cancer Center, University of Michigan School of Medicine, Ann Arbor, Michigan, USA.
⁷ Division of Pediatric Hematology, Oncology, and Stem Cell Transplantation, Department of Pediatrics, New York Medical College, Valhalla, New York, USA.
⁸ Division of Pediatric Hematology/Oncology, Department of Pediatrics, Seattle Children's Hospital, Seattle, Washington, USA.
⁹ Department of Hematology/Oncology/Bone Marrow Transplantation, Cleveland Clinic Children's, Cleveland, Ohio, USA.

PMID: 37403815
DOI: 10.1002/cncr.34942

Abstract

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.

Keywords: Ewing sarcoma; adjuvant chemoradiotherapy; consensus; metastasis; neoadjuvant therapy; stem cell transplantation.

Publication types

Review