Abstract
Extraosseous Ewing sarcoma is a malignant mesenchymal tumor. It can arise from any location; however, it generally appears in the paravertebral regions or in soft tissues of the extremities. It generally follows a rapid course and has high rates of recurrence. Pretreatment evaluation of the extension of disease is paramount since this is the most important factor in the outcome. Treatment consists of local surgery and neoadjuvant chemotherapy with some cases requiring radiotherapy. We describe the biggest tumor of EES primary to the lung parenchyma, with extensive tumor infiltration and the unique feature of airway obstruction. The tumor was successfully resected, and the patient treated with chemotherapy and radiotherapy. Extraosseous Ewing sarcoma of the lung is an extremely rare disease with less than 40 cases described in the literature.
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