Comprehensive review of the Ewing sarcoma family of tumors (ESFT) — a group of round-cell malignancies unified by shared EWS-ETS chromosomal translocations — covering epidemiology, diagnostic workup, staging, surgical and radiation decision-making, chemotherapy protocols, prognostic stratification, and future targeted therapies.
When you see a diaphyseal permeative lesion with an onion-skin periosteal reaction in an adolescent, think ESFT and initiate full staging including bone marrow biopsy — treat as systemic disease from day one regardless of apparent localization.
At resection, strive for wide margins and assess histologic necrosis in the specimen, as percent viable tumor directly predicts survival and guides further therapy intensity.
Comprehensive review of the Ewing sarcoma family of tumors (ESFT) — a group of round-cell malignancies unified by shared EWS-ETS chromosomal translocations — covering epidemiology, diagnostic workup, staging, surgical and radiation decision-making, chemotherapy protocols, prognostic stratification, and future targeted therapies.
When you see a diaphyseal permeative lesion with an onion-skin periosteal reaction in an adolescent, think ESFT and initiate full staging including bone marrow biopsy — treat as systemic disease from day one regardless of apparent localization.
At resection, strive for wide margins and assess histologic necrosis in the specimen, as percent viable tumor directly predicts survival and guides further therapy intensity.