Ewing's sarcoma family of tumors: an overview from diagnosis to survivorship

Abstract

The Ewing's sarcoma family of tumors (ESFT) is a malignant primary bone tumor often involving soft tissue that affects not only children but also young adults. Since 1992, with the addition of ifosfamide and etoposide to standard chemotherapy for primary tumors, much improvement has been made in the treatment of ESFT, with a primary focus on children. Though often recognized as a childhood cancer, it can affect individuals into the middle years of their lives, but little is known about the outcomes of adults with ESFT. ESFT, which includes Ewing's sarcoma, extraosseous Ewing's sarcoma, Askin tumor, and primitive neuroectodermal tumor, is the second most common primary malignant bone tumor in children and adolescents. It accounts for 10% of primary malignant bone tumors in children and 3% of all childhood malignancies. The most common presenting symptoms of ESFT are pain or swelling. Treatment for ESFT consists of a multimodal approach, including chemotherapy, radiation therapy, and surgery. Children and young adults with Ewing's sarcoma face many physical challenges from their illness and the complications of their treatments. Nurses play an instrumental role in assessment techniques, which lead to prompt evaluation and intervention. Nurses are vital in the education and reinforcement of supportive care needs for this patient population.