Prolonged survival with left atrial spindle cell sarcoma

Abstract

Primary spindle cell sarcoma of the left atrium is a rare tumour. Optimal treatment is to obtain complete surgical clearance of the tumour. The anatomic location of the tumour, infiltration into vital structures and difficult access provides a surgical challenge for resection of the lesion and reconstruction of the defect. The prognosis of patients with a primary cardiac sarcoma is very poor because of their resistance to treatment with chemotherapy and radiotherapy. Metastases and local recurrences are common despite optimal multimodality treatment. This report describes a 48-year-old gentleman who underwent multiple surgeries to achieve an 11-year survival since the diagnosis. The operative techniques have been described.

Primary Cardiac Sarcoma

“…4,5 The literature is largely restricted to case reports and small case series. [6][7][8][9][10][11][12][13][14] Malignant cardiac tumors have a poor prognosis with overall median survival ranging from 6 months to a few years. 5,[15][16][17][18][19][20] The most common symptom at presentation is dyspnea, whereas the complete spectrum includes but is not limited to cardiac symptoms (chest pain, syncope, pericardial effusions, valvular dysfunction, arrhythmias, congestive heart failure, thromboembolic disease, sudden cardiac death) and systemic symptoms of underlying malignant disease (eg, fatigue, fever, weight loss).…”

High-Grade Left Atrial Pleomorphic Sarcoma: Case Report and Review

“…The clinical presentation of these tumors is very heterogeneous, and the symptoms are due to compromise in ventricular afterload, arrhythmias, embolic events, and metastasis [9, 10]. The patients also can present dyspnea (48%), chest pain (22%), heart failure (13%), and pericarditis (5%).…”

Right atrial spindle cell sarcoma as a rare cause of tricuspid stenosis

“…They lack a specific histology, with negative immunohistochemistry for desmin, factor VIII and keratin. They are extremely rare in heart, with only few cases reported so far 9 10. Prognosis depends on the grade of the tumour, which is based on the mitotic activity, necrosis and degree of cellular differentiation.…”

“…Cardiac sarcomas have poor prognosis with a mean survival of <12 months. In rare cases, survival up to 11 years has been reported 10. Neoadjuvant therapy has a limited role.…”