An adolescent with primary undifferentiated pleomorphic sarcoma of the
heart with MDM2 amplification successfully treated with multimodal
therapy: Case report and Review of Literature
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.