Nupur Mittal1*, Ira Miller2, James Cameron3, Mahesh Gopalakrishnan4, Karim Diab1, Dilip Nath5 and John A Kalapurakal4
1Department of Pediatrics, Rush University Children’s Hospital, USA
2Department of Pathology, Rush University Medical Center, USA
3Department of Radiology, Rush University Medical center, USA
4Department of Radiation Oncology, Northwestern University, USA
5Department of Pediatric Surgery, Washington University, USA
Primary Undifferentiated Pleomorphic Sarcomas (UPS) of the heart are rare in the pediatric age group with an aggressive course, a dismal prognosis and no standard recommendations present for best management. Diagnosis is often difficult and late, as they may be insidious and masquerade as other benign cardiac tumors. We present a rare case of cardiac UPS with MDM2 amplification in a 13 year old girl with 3.5 year survival with complete remission following a combination of early gross total surgical resection followed by adjuvant chemoradiation therapy. The case highlights the importance of an accurate and early diagnosis and an aggressive multimodal approach for improved survival with the assistance of a multidisciplinary team.
Cardiac sarcoma; Pleomorphic undifferentiated sarcoma; Cardiac gated IMRT
Mittal N, Miller I, Cameron J, Gopalakrishnan M, Diab K, Nath D, et al. Successful Use of Multimodal Therapy for an Adolescent with Primary Undifferentiated Pleomorphic Sarcoma of Heart with MDM2 Amplification: Case Report and Review of Literature. Oncol Case Report J. 2021;4(1):1031..