Commentary
Video
Author(s):
Damon R. Reed, MD, discusses the genetic differences that distinguish Ewing sarcoma from osteosarcoma, as well as unmet needs in patients with these diseases.
Damon R. Reed, MD, division head, Pediatric Solid Tumors, chief, Pediatric Sarcoma service, Memorial Sloan Kettering Cancer Center, discusses the genetic differences that distinguish Ewing sarcoma from osteosarcoma, as well as unmet needs in patients with these diseases.
Ewing sarcoma results from approximately 6 genetic changes within the human genome, including a translocation event in which the FLI1 gene on chromosome 11 and the EWSR1 gene on chromosome 22 are fused together, Reed explains.
Conversely, thousands of genetic changes can occur in osteosarcoma, and these changes vary among patients with this disease, Reed emphasizes. Therefore, the genetic makeup of osteosarcoma is more complex than that of Ewing sarcoma, with few overlaps between individual patients, according to Reed.
Patients with osteosarcoma and Ewing sarcoma both need novel therapies, as there have been no major developments in these treatment landscapes since the early 1980s, Reed notes. Chemotherapeutic strategies in these diseases have become more refined as researchers have learned more about optimal intensities and schedules of chemotherapy, Reed says. These developments have generated incremental improvements in the treatment of patients with Ewing sarcoma over the past few decades, Reed explains. However, patients with osteosarcoma have not experienced a steady increase in treatment efficacy, and the cure rates in this disease are similar to those of 30 or 40 years ago, Reed emphasizes.
Although osteosarcoma and Ewing sarcoma are rare tumors, many researchers are passionate about managing these diseases leading to an organized and coordinated community, Reed says. Thus, new clinical trials typically enroll patients quickly. However, many of these trials do not meet their primary end points, Reed notes. Improved science and better ways of evaluating potential agents may provide the sarcoma community with more standardized approaches to treatment, Reed emphasizes. Research and disease management in this landscape needs to become more methodical to investigate why certain agents work well and how they may inspire further research, Reed explains. Additionally, combination regimens may represent effective novel treatment strategies for these patients.
Clinicians referring a patient to MSK can do so by visiting msk.org/refer, emailing referapatient@mskcc.org, or by calling 833-315-2722.