Ewing Sarcoma PDQ®

In brief

NCI's evidence-based clinical reference for healthcare professionals managing Ewing sarcoma, a rare bone and soft tissue malignancy primarily affecting children and young adults. Covers diagnostic approaches, multimodal treatment strategies including chemotherapy, surgery, and radiation, and prognostic factors for this aggressive tumor.

• Ewing sarcoma is a rare bone and soft tissue malignancy requiring multimodal treatment with chemotherapy, surgery, and/or radiation.
• Most common in adolescents and young adults; presents with pain, swelling, and systemic symptoms like fever.
• Diagnosis requires biopsy with molecular confirmation of EWSR1 gene rearrangement (pathognomonic translocation).
• Treatment involves neoadjuvant chemotherapy followed by local control (surgery preferred over radiation when feasible).
• Prognosis depends on tumor size, location, metastatic status at diagnosis, and response to initial chemotherapy.

Written by the GCMD Library team from the guideline.