Overview of the Ewing’s Sarcoma Family of Tumors
The Ewing’s family of tumors includes Ewing’s sarcoma of bone, non-bone Ewing’s sarcoma, peripheral primitive neuroectodermal tumors (PNET) and Askin’s tumor (PNET of the chest wall). The incidence of Ewing’s in children and adolescents is 2.1 per million.1 Ewing’s sarcomas occur most frequently in the second decade of life and account for three to four percent of childhood and adolescent cancers. However, Ewing’s sarcoma can occur at any age, even in the very elderly. Ewing’s sarcoma of the bone most commonly involves the arms and legs, pelvis, chest and spine, and skull. Non-bone Ewing’s sarcoma most commonly involves the trunk, extremities, head and neck, and retroperitoneum (the area outside or behind the tissue that lines the abdomen) Common sites for PNET are the chest, abdomen/pelvis, extremities, and head and neck.
Cancer in children and adolescents is rare. Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team incorporates the skills of the primary care physician, pediatric surgical sub-specialists, radiation oncologists, pediatric oncologists/hematologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others in order to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life.2 At these pediatric cancer centers, clinical trials are available for most of the types of cancer that occur in children and adolescents, and the opportunity to participate in these trials is offered to most patients and families. Clinical trials for children and adolescents with cancer are generally designed to compare potentially better therapy with therapy that is currently accepted as standard. The majority of the progress made in identifying curative therapies for childhood cancers has been achieved through clinical trials.
Evidence suggests that roughly 75 percent of patients with Ewing’s sarcoma present with localized disease, and that 80 percent or more of these patients may be curable with combined modality therapy with chemotherapy, radiation therapy and surgery.3 For those who present with metastatic disease the cure rate is 20 to 30 percent.4 These figures represent results from the most recent treatment strategies which are significantly better than in the past.
For details about the treatment of Ewing’s sarcoma, click on the appropriate stage:
Localized Ewing’s sarcoma: Localized Ewing’s sarcoma affects only the bone in which it developed and the tissues next to the bone, such as muscle and tendon. There is no detectable spread of the cancer to other areas of the body.
Metastatic Ewing’s sarcoma: Ewing’s sarcoma that has spread from the initially affected bone to one or more sites in the body, distant from the site of origin, is called metastatic.
Recurrent Ewing’s sarcoma: Ewing’s sarcoma that has not responded to treatment or has returned after an initial response to treatment is considered recurrent.
1Arndt CAS and Crist WM. Common musculoskeletal tumors of childhood and adolescence. Medical Progress. New England Journal of Medicine 1999;341:242-352.
2Bernstein M, Kovar H, Paulussen M, et al. Ewing’s sarcoma family of tumors: current management. Oncologist 2006;11:503-519.
3Krasin MJ, Rodriguez-Galindo C, Davidoff AM, et al. Efficacy of combined surgery and irradiation for localized sarcoma family of tumors. Pediatric Blood Cancer 2004;43:229-236.
4Holcombe EG, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. The New England Journal of Medicine 2003;348:694-701.