Frank's Sign Coexisting With a Giant Right Atrial Myxoma: A Case Report

Frank's sign, a diagonal earlobe crease linked to atherosclerotic disease, remains poorly understood, and no association with cardiac tumors has been reported. We describe the case of a 68-year-old man who presented with exertional dyspnea and an elevated N-terminal pro-B-type natriuretic peptide level of 2,251 pg/mL. Transthoracic echocardiography identified a 75 mm × 50 mm pedunculated mass in the right atrium that prolapsed toward the tricuspid valve. Frank's sign was present bilaterally despite the absence of coronary or peripheral arterial disease. Contrast-enhanced CT confirmed attachment of a gelatinous mass to the atrial free wall, and coronary CT angiography demonstrated no stenosis. The tumor was excised via median sternotomy under cardiopulmonary bypass established with aorto-superior vena cava cannulation, supplemented by a femoral venous cannula to facilitate inferior vena cava drainage. Histology showed spindle cells in a myxoid matrix without atypia, consistent with benign myxoma. To our knowledge, this is the first report describing the coexistence of Frank's sign and cardiac myxoma. We speculate that tumor-derived cytokines such as interleukin-6 and vascular endothelial growth factor might impair earlobe microcirculation; however, coincidental coexistence cannot be excluded.