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An adolescent with primary undifferentiated pleomorphic sarcoma of the heart with MDM2 amplification successfully treated with multimodal therapy: Case report and Review of Literature
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  • Nupur Mittal,
  • Ira Miller,
  • James Cameron,
  • Dilip Nath,
  • Karim Diab,
  • Mahesh Gopalakrishnan,
  • John Kalapurakal
Nupur Mittal
Rush University Medical Center
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Ira Miller
Rush University
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James Cameron
Rush University Medical Center
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Dilip Nath
Washington University in St Louis
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Karim Diab
Rush University Medical Center
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Mahesh Gopalakrishnan
Northwestern Memorial HealthCare Corp
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John Kalapurakal
Northwestern University
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Abstract

Primary Undifferentiated pleomorphic sarcomas (UPS) of the heart extremely rare but aggressive tumors in the pediatric age group with an extremely poor prognosis and no clear guidelines available for best management. Diagnosis is often delayed, as they may be insidious and masquerade as other cardiac benign tumors. We present a rare case of cardiac UPS in a 13 year old with 40 months disease free survival following a combination of early gross total surgical resection followed by adjuvant chemotherapy and radiation therapy. The case highlights the importance of a timely diagnosis and multimodal multidisciplinary aggressive approach for improved survival.