Surgical Management of Recurrent Leiomyosarcoma in Heart
A 63-year-old woman with an impression of a recurrent tumor was admitted with a chief complaint of shortness of breath 10 months after surgery for cardiac leiomyosarcoma. In her past medical history, she had undergone surgery for the removal of leiomyosarcoma in the main pulmonary artery at a different hospital, where she had been admitted with a chief complaint of dyspnea (New York Heart Association Functional Classification II-III). In addition, she did not have hypertension or diabetes mellitus, other than the previous cardiac surgery.\nUpon admission, her blood pressure was 130/90, and her heart rate was 90 beats/min showing a sinus rhythm. Her height, body weight, and body mass index were 160 cm, 60 kg, and 24.4 kg/m2, respectively. The blood test taken upon her admission showed creatine kinase-MB 2.5 and troponin-I <0.02, which were within normal levels. Arterial blood gas analysis revealed pH 7.48, PO2 61.3 mmHg, PCO2 27.2 mmHg, and O2 saturation 92.3%.\nIn her chest X-ray, no cardiomegaly was observed, but patchy haziness of the right middle lobe was seen. The cardiac computed tomography (CT) showed a massive amount of thromboembolism in the pulmonary trunk, right pulmonary artery, and left anteromedial basal segment artery (). Lung cancer, pulmonary infarction, myocardial infarction, and coronary artery disease were not observed. Because of a suspicion of recurrent leiomyosarcoma, a surgical resection was decided upon (preoperative echocardiogram was not carried out). There was no evidence of lung metastasis in CT; therefore, lobectomy of the lung was not considered.\nAfter carrying out median sternotomy, cardiopulmonary bypass was initiated. Transverse pulmonary arteriotomy was performed in the area straight above the pulmonary valve. Then, a mass with a volume of 4×4×3.5 cm hanging on the pulmonary valve with a stalk was observed. The mobile gelatinous mass was hanging loosely in the right ventricular outflow track (RVOT), infundibulum, and main pulmonary artery. The incision of the right pulmonary artery was made up to the superior vena cava. An incision of the left pulmonary artery was extended to the first bifurcation area. Then, the myxoid mass was resected (). The sufficient back flow from the distal pulmonary artery was verified in both the right and the left pulmonary arteries. The harvested greater saphenous vein graft was used for the reconstruction and widening of the main pulmonary artery and left pulmonary artery. Because the myxoid mass had a well-defined capsule and stalk, we thought that there was no invasion to the nearby apparatus; therefore, we decided upon a pulmonary valve preservation operation.\nThe duration of extracorporeal circulation was 235 minutes, while that of aortic cross clamping was 85 minutes. Extubation was carried out 12 hours after surgery, and the patient was transferred to a general ward on the fifth postoperative day. Cardiac CT performed on the seventh postoperative day confirmed that there was no remnant mass in the pulmonary trunk, RVOT, or in either of the pulmonary arteries (). The patient was discharged on the fourteenth postoperative day without any complications.\nThree months after hospital discharge, she again developed dizziness and palpitation, and an echocardiogram was carried out and showed a generally hypoechoic and heterogeneous mass. The CT images showed a 2.5×2.8 cm mass at the base of the interventricular septum of the RVOT, as well as redundant movements in the direction of the pulmonary artery (). As anticipated, the right ventricular systolic pressure increased to 70 to 75 mmHg. Upon the suspicion of recurrent cardiac leiomyosarcoma, we decided on a radical resection of the tumor.\nTransverse pulmonary arteriotomy under cardiopulmonary bypass was performed in the area directly above the pulmonary valve. A gelatinous capsular mass causing RVOT obstruction was removed in the pulmonary valve and subvalvar infundibulum. Then, the transvalvar RVOT reconstruction was performed using a Prima plus 25 mm valve, and the vascular graft after the pulmonary valve was removed. The duration of extracorporeal circulation was 117 minutes. The postoperative course was uneventful, and an echocardiogram performed on the sixth postoperative day did not show a mass in the RVOT and revealed a well-functioning pulmonic valve. The images of cardiac CT carried out on the eighth postoperative day showed no residual mass of the pulmonary trunk, RVOT, or in either of the pulmonary arteries (). The patient was discharged from the hospital on the tenth postoperative day.\nDuring the follow-up six months after hospital discharge, a chest CT disclosed findings that leiomyosarcoma metastasized to left upper lobe (lingular segment) and left lower lobe (). Lingular segmentectomy and lower lobectomy were decided upon and performed under thoracotomy. A tumor 3×3×3 cm in size, dwelling apart from the pulmonary artery, was observed in the lingular segment of the left upper lobe. Another tumor mass with a size of approximately 3.5×3×3 cm was found in the anterior basal segment of the left lower lobe, for which lingular segmentectomy and lower lobectomy were carried out. The patient was discharged from the hospital 10 days after surgery. She has been asymptomatic and free of recurrence for 36 months after the last operation.