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Complete resection of undifferentiated cardiac sarcoma and reconstruction of the atria and the superior vena cava: case report
© Furukawa et al.; licensee BioMed Central Ltd. 2012
Received: 8 May 2012
Accepted: 23 September 2012
Published: 27 September 2012
Primary cardiac tumors are rare with an incidence ranging from 0.001% to 0.03% in autopsy series. The prognosis of cardiac sarcomas remains poor because it proliferates rapidly and distant metastases are often found at diagnosis. A 47-year-old male complained of persistent cough. The chest roentgenogram was normal. Subsequent computed tomography revealed a mass in the right atrium. Echocardiography and magnetic resonance imaging confirmed also a right atrial mass (34 x 35 mm) infiltrating the atrial septum. The tumor was completely resected en bloc, including the anterior and lateral right atrial walls, the left atrial dome, and a large segment of the superior vena cava, and reconstructed the atria and superior vena cava with bovine pericardium. The tumor was histologically and immunohistochemically diagnosed as undifferentiated pleomorphic sarcoma. This type of cardiac sarcoma is very rare and usually found in the left atrium. Twenty-seven months after surgery, the patient is doing well without metastasis or local tumor recurrence.
Primary cardiac tumors are uncommon (incidence in autopsy series, 0.001–0.03%). Among them, malignant neoplasms account for <25%. The prognosis of cardiac sarcomas remains poor. Although a more effective therapy would be desirable, complete resection is the current standard treatment in cases without metastases. We describe a case of undifferentiated pleomorphic sarcoma infiltrating both atria and the superior vena cava. We removed the tumor completely, including the sinus node, by en bloc resection and reconstructed the atria and superior vena cava with bovine pericardium.
The patient recovered without complications, was discharged home on postoperative day 7 and referred to undergo local radiotherapy and systemic chemotherapy with ifosfamide and paclitaxel. For a duration of three months, the patient was anticoagulated with warfarin and then switched to oral acetyl salicylic acid. Thirty-five months after surgery, the patient is doing well and remains free of metastases or local tumor recurrence.
Primary cardiac tumors are uncommon. Approximately three fourth of them are malignant. Sarcomas comprise two-thirds of these malignancies. Cardiac sarcomas include angiosarcoma; sarcoma with myofibroblastic differentiation; synovial sarcoma; and rhabdomyosarcoma. Sarcomas with myofibroblastic differentiation are the most diverse types, subclassified as undifferentiated pleomorphic sarcoma; osteosarcoma; leiomyosarcoma; and fibrosarcoma. In several large series[4, 5], the most common type was angiosarcoma, while undifferentiated pleomorphic sarcomas were relatively rare (only 6–7%). Undifferentiated pleomorphic sarcoma is usually found in the left atrium and exhibits a solid and infiltrative pattern. As described here, undifferentiated pleomorphic sarcoma with bilateral involvement is very rare.
The prognosis of patients with cardiac sarcoma is poor because the tumor proliferates rapidly and distant metastases are often found at the time of the initial diagnosis. Hamidi et al. reported that the median survival of patients who underwent surgery was 12 months, whereas survival of patients not operated upon was only 1 month. Simpson et al. reported that the median survival of patients with and without metastasis was 5 and 15 months, respectively.
In our case, the tumor infiltrated the left atrium and approximately two-thirds of the right atrium, involving the sinus node and superior vena cava. Fortunately, this infiltration was relatively anterior in the left atrial dome, facilitating complete resection and repair with bovine pericardium. When the tumor infiltrates the left atrium more deeply and to a greater extent, resection and reconstruction is relatively difficult. In such cases, autotransplantation is an option permitting complete resection and repair[2, 7].
Cardiac sarcomas are often asymptomatic. In advanced cases, they occasionally produce nonspecific symptoms. Malignant cardiac tumors with direct invasion of the pericardium can cause pericardial effusion or arrhythmias, such as atrial fibrillation, heart block, or recurrent ventricular tachycardia[2, 3]. We performed en-bloc resection of the tumor, including the sinus node. Simultaneously, permanent epicardial pacemaker leads were placed onto the remaining right atrium and onto the left ventricle. Radical resection of the tumor is difficult or impossible if the tumor infiltrates the conduction system. In many cases, cardiac sarcoma is so widespread that only palliative surgery can be performed. It remains unclear if there is an alternative to radical surgical resection in patients without metastatic disease. Because cardiac sarcoma is uncommon and because there are only a few limited studies on the subject, no standard treatment has been defined. Although aggressive resection of cardiac sarcoma is challenging, some studies clearly demonstrate that complete tumor removal is the best method for improving survival[1, 2, 4].
In our case, chemotherapy and radiotherapy were initiated relatively early after surgery, especially in view of reports[2, 7] of local recurrences or distant metastases several years after successful resection. The effectiveness of such adjuvant therapies for cardiac sarcoma remains controversial. However, studies[1, 2] have shown that combining several carcinostatics is more effective than single-agent therapy, and that postoperative radiotherapy can improve survival.
We presented a rare case of undifferentiated pleomorphic cardiac sarcoma successfully removed by en-bloc resection which included two-thirds of the right atrium, the left atrial dome and a large segment of the superior vena cava, reconstructing the defects with bovine pericardium. Despite successful management, close follow-up is mandatory because of the overall poor prognosis of cardiac sarcoma.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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