Skeletal Ewing sarcoma

Skeletal Ewing sarcoma (also known as Ewing sarcoma of bone or osseous Ewing sarcoma) is a rare, aggressive malignant bone tumor that belongs to the Ewing sarcoma family of tumors (ESFT). It is the second most common primary bone cancer in children and young adults, predominantly affecting individuals between the ages of 5 and 25 years. The tumor most commonly arises in the long bones of the legs and arms (particularly the femur, tibia, and humerus) as well as the pelvis, ribs, and vertebrae. Skeletal Ewing sarcoma is characterized by a specific chromosomal translocation, most frequently t(11;22)(q24;q12), which produces the EWSR1-FLI1 fusion gene found in approximately 85% of cases. Key symptoms include localized bone pain that may worsen at night or with activity, swelling and tenderness at the tumor site, and a palpable mass. Patients may also experience systemic symptoms such as fever, fatigue, weight loss, and elevated inflammatory markers. Pathologic fractures can occur at the site of the weakened bone. At diagnosis, approximately 20-25% of patients present with metastatic disease, most commonly to the lungs, other bones, or bone marrow. The current treatment approach involves a multimodal strategy combining neoadjuvant (pre-surgical) chemotherapy, local control through surgery and/or radiation therapy, and adjuvant (post-surgical) chemotherapy. Standard chemotherapy regimens typically include vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide (VDC/IE protocol). With modern treatment protocols, the five-year survival rate for localized disease is approximately 60-70%, while metastatic or recurrent disease carries a significantly poorer prognosis. Research into targeted therapies and immunotherapy approaches is ongoing to improve outcomes, particularly for patients with relapsed or refractory disease.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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