Primary Myxofibrosarcoma of the Left Atrium: Case Report and Review of the Literature

Abstract

A 63-year-old woman with progressive dyspnea underwent transthoracic echocardiography and was found to have a large multilobed mass in the left atrium that was attached to lateral wall. On inspection during surgery, the tumor was found to infiltrate the posterior mitral annulus and leaflet. The patient underwent surgical resection of the tumor and mitral valve replacement. Histologic and cytochemical evaluation confirmed that the tumor was a myxofibrosarcoma. Despite chemotherapy, the tumor recurred and the patient died 3 months after surgery.

State of the art paper Sarcomas of the heart as a difficult interdisciplinary problem

“…It requires an extensive excision with a wide healthy tissue margin. High-grade sarcomas give metastases generally to the lymph nodes, lungs, bones or the brain [53]. Fibrosarcomas rarely involve the lymph nodes.…”

“…Ultrastructural examination confirmed its fibroblastic etiology. In the literature there are only 7 cardiac myxofibrosarcoma cases reported, including 4 in the left atrium [53]. …”

Primary intermediate-grade cardiac myxofibrosarcoma with osseous metaplasia: an extremely rare occurrence with a previously unreported feature

“…Cardiac MFS however is exceedingly rare, and the cases reported concern the left Cardiovascular Pathology 23 (2014) 376-378 atrium, with only one case reported in the right ventricle [6]. The majority appear in women, and dyspnea is the most common clinical symptom [7,8]. Histopathology examination reveals fibroblastic differentiation and extensive myxoid stroma with prominent blood vessels displaying an arborizing pattern, cellular atypia and nuclear pleomorphism.…”

Primary malignant peripheral nerve sheath tumour of the heart

“…Currently, all research on cardiac sarcomas report an improved rate of survival after the tumor is radically removed. In addition, it is usually required to for the reconstruction of cardiac chambers [10][11][12][13][14][15]. Therefore, this is best achieved when tumors are encapsulated and well demarcated [16][17][18][19].…”