Left atrial myxofibrosarcoma: Diagnostic approach through imaging techniques

Abstract

Primary cardiac sarcomas are rare. A 63-year-old woman presented with progressive symptoms of fatigue, palpitations, and dyspnea. Transthoracic echocardiography detected a mass in the left atrium, fixed and probably attached to the interatrial septum suggestive of myxoma. Transesophageal echocardiography confirmed the existence of a large lobulated mass in the left atrium measuring 45 mm × 25 mm in diameter. Subsequent cardiac magnetic resonance imaging showed a mass of heterogeneous appearance, with broad base of implantation on the posterior wall of the left atrium. The extension study with multidetector computed tomography showed superficial infiltration of the atrial wall, without involvement of the mitral valve or pulmonary veins and no extension to other extracardiac structures. The patient underwent surgery resecting a bilobed mass, smooth and yellowish, strongly attached to the posterior wall of left atrium. The pathologic study was consistent with the diagnosis of myxofibrosarcoma. <Learning objective: Primary cardiac tumors are among the most challenging disease entities to diagnose because of their rarity and highly variable and usually nonspecific clinical presentation. Although the definitive diagnosis of a cardiac tumor, whether benign or malignant and the tumor type is provided by histological examination, imaging techniques are essential for confirming the existence of a cardiac mass and characterizing the tumor before obtaining a sample for pathologic examination.>

Left atrial appendage myxofibrosarcoma: A rare masquerader of myxoma and thrombus—“all that glitters is not gold”

“…It follows, if there are no constitutive signs and symptoms (as in our patient), differentiating between these 2 masses (tumor vs thrombus) can become difficult . Left atrial myxofibrosarcoma may simulate LA myxoma in its clinical presentation, as is true in our case and via TTE where the patient had presentation similar to an obstructive myxoma.CMR can add diagnostic information because of its capability of noninvasive tissue characterization if performed in addition to echocardiography . However, in this case because of the large size of the mass, its origin from the LAA, areas of “necrosis,” and most importantly the extreme rarity of cardiac primary myxofibrosarcoma, it was extremely difficult to diagnose with the available noninvasive imaging techniques.…”

“…On occasion, the specificity for routine echocardiography diagnosis for LAA masses, typically >99% due to the pretest probability and prevalence, can be misleading. We note that angiosarcoma constitutes the most common variety of primary malignant neoplasm of the heart typically >90% arising from the LA. Myxofibrosarcoma, as in our case, rarely arises in the heart, and when it does, it most frequently affects the left atrium, never the LAA.…”

Myxofibrosarcoma of the heart: A case report with positive pleural effusion cytology

“…Of these, 76.5% were benign cardiac tumors, and 23.5% were malignant, and of the malignant tumors, onethird were angiosarcomas, 20% were rhabdomyosarcomas, 15% were mesotheliomas, and 10% were fibrosarcomas. [5][6][7][8][9] Myxofibrosarcomas are most commonly found in the left atrium, but have been reported to arise in all chambers. 11 Primary cardiac myxofibrosarcomas are extremely rare.…”

“…3 Fibrosarcoma is a rare cardiac tumor, representing about 5% of AFIP cases and recent series of cardiac sarcomas resected at surgery. [5][6][7][8][9] Here, we present a case of primary cardiac myxofibrosarcoma in which pleural effusion cytology and imprint cytology specimens were obtained at resection and autopsy. [5][6][7][8][9] Here, we present a case of primary cardiac myxofibrosarcoma in which pleural effusion cytology and imprint cytology specimens were obtained at resection and autopsy.…”

“…4 Myxofibrosarcoma is a variant of fibrosarcoma, and only 22 cases of cardiac myxofibrosarcoma have been reported in the literature. [5][6][7][8][9] Here, we present a case of primary cardiac myxofibrosarcoma in which pleural effusion cytology and imprint cytology specimens were obtained at resection and autopsy. Immunocytochemistry with a cell transfer technique of the pleural fluid and imprint cytology specimens were performed.…”