Left atrial spindle cell sarcoma: a case report

Abstract

Background Primary cardiac spindle cell sarcomas are extremely rare with only a few cases reported. They are frequently misdiagnosed on cardiac magnetic resonance (CMR) imaging as benign myxoma or thrombi and the suspicion of a malignant sarcoma arises only during surgery. This case report describes a case of cardiac spindle cell sarcoma diagnosed after surgery, where the initial diagnostic possibilities included an intramural thrombus and a cardiac myxoma. Case summary A 57-year-old woman was referred to our hospital for evaluation of a possible recurrent myxoma in the left atrium on echocardiography. Cardiac magnetic resonance imaging confirmed these masses as mural thrombotic masses, with a possible remnant of myxoma. After 2 months of anticoagulation therapy, the masses did not decrease in size on CMR imaging, and surgical removal was indicated. The atrial masses were surgically resected together with a large part of the left atrium. Histological examination showed spindle cell sarcoma. Unfortunately, the resection margins were positive and it was not possible to remove more atrium. PET-CT revealed metastasis in the right femur. The patient passed away 1 year after surgery. Discussion The rarity of spindle cell sarcoma and its similarities to benign cardiac myxoma and thrombi on echocardiography and CMR imaging present a diagnostic challenge when evaluating patients pre-operatively. Therefore, a malignant spindle cell sarcoma may only be diagnosed during surgery, after histological examination.

Left atrial spindle cell sarcoma

“…A summary of other cases reported previously and our case are shown in Table 1 . [ 3 – 9 ] The tumor usually infiltrates the left atrium, as seen in our patient. Lung metastases (40%) and extrathoracic metastasis (20%) to kidneys, lymph nodes, brain, and skin are often seen.…”

Case report: The diagnostic challenge of primary cardiac intimal sarcoma

“…Primary cardiac intimal sarcoma is an extremely rare subtype of cardiac tumor, with only a few such cases having been reported (1)(2)(3)(4)(5)(6)(7)(8). It often occurs in the left atrium, whereas it seldom originates in the right atrium and mitral valve (6,8).…”