Right ventricular undifferentiated pleomorphic sarcoma: A case report

Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, is a neoplasm that occurs most often in the extremities, trunk, and retroperitoneum. Rarely, UPS can occur in the cardiac chambers and great vessels. The diagnosis of UPS is difficult to establish with noninvasive imaging techniques, and these tumors may be mistaken for benign neoplasms preoperatively. Surgical excision is the standard therapy, although the extent and location of the tumor may limit the ability to perform a complete resection. Adjuvant chemotherapy and/or radiation are often used for incomplete resections. We report the case of a 57-year-old woman with a large right ventricular UPS who presented with signs of right-sided heart failure. Preoperative imaging was suggestive of a myxoma; however, histopathologic evaluation of the specimen confirmed a diagnosis of UPS. Microscopic margins of the specimen were positive, and adjuvant chemotherapy was given. We discuss diagnostic and treatment considerations for this unusual cardiac tumor. <Learning objective: The diagnosis and treatment of primary cardiac tumors are challenging. Noninvasive imaging often lacks the sensitivity and specificity to differentiate between benign and malignant neoplasms. In addition, the extent and location of tumor involvement may limit the ability to perform a complete resection. The diagnosis and treatment of right ventricular undifferentiated pleomorphic sarcoma are discussed.>.