BACKGROUND: Primary cardiac tumors are rare and mostly benign. Cardiac sarcomas are the most common malignant neoplasms of the heart and harbor a dismal prognosis of 6 to 12 months. The diagnosis of cardiac sarcomas may be challenging. Treatment entails surgical resection despite the high rate of recurrence, as well as adjuvant chemotherapy.

CASE REPORT: In this report, we discuss a case of a 58-year-old male with undifferentiated pleomorphic primary cardiac sarcomas who received multiple lines of treatment that included surgery, chemotherapy, and targeted therapy and was alive more than 4 years after his diagnosis. Herein, we discuss the different treatment regimens utilized and we present detailed imaging of his case findings at different treatment stages.

CONCLUSIONS: Treatment of undifferentiated pleomorphic cardiac sarcoma requires a multidisciplinary approach. Surgery and adjuvant treatment are commonly utilized, while neoadjuvant treatment is under investigation.

Keywords: Cardiac Surgical Procedures, Chemoradiotherapy, Adjuvant, Sarcoma, Chemotherapy, Adjuvant, Heart Neoplasms, Histiocytoma, Malignant Fibrous, Neoadjuvant Therapy, Time Factors