Unresectable mediastinal soft tissue sarcomas are often aggressive and associated with a poor prognosis. A 17-year-old male presented with progressive fatigue, shortness of breath, and heart palpitations secondary to an extensive mass involving the mediastinum and pericardium. He was treated with chemotherapy per protocol Children's Oncology Group Protocol ARST0332 and proton beam therapy to the involved mediastinum, pericardium, and heart. At the 5-year follow-up evaluation, he remained disease-free on surveillance imaging. An echocardiogram revealed a 55% to 60% left ventricular ejection fraction. Given the patient's extended survival, we present the oncologic rationale for treatment and considerations of late toxicity.
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