Circ J 2009; 73: 779 -782 rimary cardiac malignant tumors are very rare and account for a quarter of malignant cardiac tumors. [1][2][3] Sarcomas constitute almost all (95%) of the primary cardiac malignancy. 3,4 We report herein a middle-aged man who presented with dyspnea and had a discordantly huge leiomyosarcoma in the right ventricle (RV) that was elongated into the right atrium (RA) and the main pulmonary artery (MPA) through the tricuspid and pulmonary valves, respectively, with impending obstruction of inflow and outflow tracts of the RV.
Case ReportA 45-year-old man visited the emergency room because of progressive shortness of breath for 1 month. He had no medical history and had been doing well without any constitutional symptoms. His blood pressure was 110/60 mmHg, pulse rate 94 beats/min, body temperature 36.7°C, and he had a respiration rate of 22 breaths/min. A physical examination revealed a healthy general appearance and systolic murmur (GIII/GVI) along the left upper parasternal border. An electrocardiography showed a heart rate of 96 beats/min with a S1Q3T3 sign, T wave inversion on the right precordial leads, and an incomplete right bundle branch block implying an acute pressure and volume overload of the RV (Figure 1). On laboratory study, the level of serum pro-B type natriuretic peptide was elevated up to 3,783 pg/ml. Other cardiac enzymes were within normal ranges. Mild thrombocytopenia (126×10 9 /L), prolonged prothrombin time (13.5 s), elevated alanine aminotransferase (57 U/L), and small ascites were also found.Transthoracic and transesophageal echocardiography (TTE/TEE) showed an 8×4 cm-sized, huge tumor that fully filled the RV and extended into the MPA through the RV outflow tract and into the RA through the tricuspid valve (TV; Figures 2A,C). The tumor had a broad-based stalk and was attached to the RV apex and interventricular septum (IVS). A Color Doppler study showed the near obstruction of inflow and outflow tracts of the RV caused by the huge tumor (Figures 2B,D). The apical anterior wall of the RV was thickened and showed partial indentation with increased echogenicity and a small amount of pericardial effusion around the RV apex. The left ventricle showed a D-shape becaues of the compression effect of the bulky tumor shifting the IVS to the left side. A cardiac magnetic resonance imaging (CMR) with gadolinium enhancement disclosed the huge RV tumor with a low-signal intensity similar to a myocardium on a T1-weighted image, and a mild highsignal intensity on a T2-weighted image (Figures 3A-C). The tumor showed heterogeneous enhancement at the delayed phase, especially at the proximal portion of the tumor attached to the RV apex ( Figure 3D).The patient underwent urgent surgery on the third hospital day to relieve the obstruction. During the cardiopulmonary bypass, the RV was not collapsed owing to the large intracavitory mass. The smooth-surfaced hard tumor originated from the RV apex and IVS, and protruded into the RA and MPA. The tumor was markedly adhered to the entire...