Is It Time to Call Time on Bone Marrow Biopsy for Staging Ewing Sarcoma (ES)?

Abstract

Simple Summary Ewing sarcoma (ES) is a rare primary bone cancer, usually found in children and adolescents, which can spread to the lungs, other bones and less commonly, the bone marrow. An accurate determination of the disease spread at baseline (staging) is important to establish prognosis, monitor treatment response and help with management decisions. There is no standard of care for staging ES, although the invasive bone marrow biopsy has traditionally been used to establish whether patients have bone marrow infiltration. Imaging techniques, including FDG-PET/CT and whole-body MRI (WB-MRI), have become established in staging other cancers with expanding use for staging ES. A number of studies have validated the accuracy, sensitivity and specificity of these modalities for detecting bone and bone marrow metastases in ES. The main aim of this review was to examine the current literature for the use of FDG-PET/CT and WB-MRI in staging ES to determine whether a bone marrow biopsy is still needed and would influence the management of patients. Hereafter, a new staging algorithm for ES recommends WB-MRI and/or FDG-PET/CT without bone marrow biopsy as the standard of care for staging localised and metastatic ES. Abstract Primary malignant bone sarcomas are rare and Ewing sarcoma (ES), along with osteosarcoma, predominates in teenagers and young adults. The well-established multimodality treatment incorporates systemic chemotherapy with local control in the form of surgery, with or without radiation. The presence and extent of metastases at diagnosis remains the most important prognostic factor in determining patient outcome; patients with skeletal metastases or bone marrow infiltration having a significantly worse outcome than those with lung metastases alone. There is, however, no accepted staging algorithm for ES. Large cooperative groups and national guidelines continue to advocate bone marrow biopsy (BMB) for staging but functional imaging techniques, such as 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) with computerised tomography (CT) have been increasingly used for staging cancers and whole-body magnetic resonance imaging (WB-MRI) for staging skeletal metastases. This review outlines the current literature, from which we conclude that BMB is no longer required for the staging of ES as it does not influence the standard of care management. BMB may, however, provide prognostic information and insights into the biology of ES in selected patients on prospective clinical trials.