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 . upon 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 / m , 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% . in 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 ( fig . 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 . after carrying out median sternotomy , then , a mass with a volume of 443.5 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 . 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 . the 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 ( fig . three 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 2.52.8 cm mass at the base of the interventricular septum of the rvot , as well as redundant movements in the direction of the pulmonary artery ( fig . 3a ) . 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 . transverse 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 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 ( fig . the patient was discharged from the hospital on the tenth postoperative day . during 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 ( fig . 4 ) . lingular segmentectomy and lower lobectomy were decided upon and performed under thoracotomy . a tumor 333 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 3.533 cm was found in the anterior basal segment of the left lower lobe , for which lingular segmentectomy and lower lobectomy were carried out . she has been asymptomatic and free of recurrence for 36 months after the last operation . leiomyosarcoma is a rare tumor that develops in the smooth muscle cells and may occur systemically anywhere . however , this tumor , which comprises 8% of all sarcomas , largely occurs in the uterus , retroperitoneum , and intra - abdominal region [ 1 - 5 ] . davidsohn was the first to report a case of leiomyosarcoma in 1908 , and cardes et al . the clinical symptoms of leiomyosarcoma in the heart or lung include chest pain and shortness of breath , which are similar to those of pulmonary embolism . chest x - ray and ct findings are very similar to those of these two disorders , making it difficult to decide upon a diagnosis . transthoracic and transesophageal cardiac echography , as well as ct of the heart , have been used as the standard methods for the diagnosis of leiomyosarcoma that infiltrates the great vessels of the heart and pulmonary artery . however , recent utilization of a diagnostic approach with electrocardiogram - guided magnetic resonance imaging has increased diagnostic sensitivity . leiomyosarcoma that invades the heart is often discovered in the left atrium , but a tumor may also be found in the right heart structures , such as the pulmonary valve and rvot on relatively rare occasions . treatment approaches to leiomyosarcoma are only surgical resection and adjuvant therapy after surgery . with respect to surgical treatment , radial excision and aggressive excision are possible . a lower recurrence rate of leiomyosarcoma has been reported with the latter approach , but the necessity of further operations to a damaged heart , such as valve replacement , pulmonary trunk , and rvot reconstructions , increase the rates of postoperative complications . either surgical resection only or adjuvant therapy after surgical resection shows an incredibly poor prognosis with a mean survival rate of 12 to 16 months after surgery . an adjuvant therapy with doxorubicin , a chemotherapeutic agent , may extend the mean survival rate to 24 months , but will not change the natural history of leiomyosarcoma . the result of pulmonary arterial repair with a greater saphenous vein graft and prima plus - utilized pulmonary valve replacement showed no recurrences within the heart during 36 months of follow - up observations at the outpatient department . regarding lung metastasis that supposedly occurred through the pulmonary vessels , a vigorous treatment attempt such as lobectomy was carried out with no current evidence of recurrence during the outpatient follow - up . in this case , the patients underwent heart surgery three times . after the third heart operation , the patient has remained alive for 36 months without recurrence of leiomyosarcoma . if a radical operation were done in the second heart operation , the third heart operation might not be required . however , if a surgeon decides that surgical resection is possible , resection of leiomyosarcoma seems to be helpful in improving the prognosis . thus , we report surgical experiences of multiple recurrences of leiomyosarcoma in cardiovascular and respiratory organs in this 63-year - old patient .
leiomyosarcoma may occur anywhere in the body but rarely occurs in the heart or great vessels . 
 leiomyosarcoma may be managed by surgical resection with or without chemotherapy or radiotherapy . 
 owing to the high rate of metastasis and poor prognosis , a definitive treatment modality for leiomyosarcoma has not yet been suggested . 
 this case study reports the surgical management of the recurrent leiomyosarcoma of the heart and the great vessels in a 63-year - old woman .