Pleomorphic rhabdomyosarcoma of the left atrium mimicking myxoma

Primary rhabdomyosarcoma (RMS) of the heart is a rare malignant tumor which has poor prognosis and survival despite surgery and adjuvant chemotherapy. The preoperative diagnosis is often difficult in view of nonspecific clinicoradiological findings. This report describes a case of a 60-year-old woman who was clinically diagnosed as left atrial myxoma. A diagnosis of pleomorphic RMS was made on histopathology after excision. Our case discusses the clinicopathological features and treatment options of cardiac RMS emphasizing the fact that histopathology and immunohistochemistry are essential to confirm the diagnosis of such an aggressive malignant tumor.