Abstract
BACKGROUND: Primary cardiac tumors are exceedingly rare, accounting for less than 0.2% of cases in autopsy series. Myxomas represent the majority, while hemangiomas are exceptionally uncommon, accounting for less than 2% of benign cardiac tumors. Their rarity, nonspecific clinical presentation, and overlapping imaging features make preoperative diagnosis particularly challenging. CASE PRESENTATION: We report the case of a 47-year-old woman with exertional dyspnoea and a large right atrial mass initially suggestive of myxoma on transthoracic echocardiography. Coronary angiography revealed a small fistulous connection, raising suspicion of a vascular lesion. Surgical resection was performed, and histopathological examination confirmed a cavernous hemangioma. The postoperative course was favorable, with no residual mass on follow-up imaging. DISCUSSION: Cardiac hemangiomas are often misdiagnosed as myxomas due to similar clinical and echocardiographic appearances. This case illustrates the diagnostic challenges posed by cardiac hemangiomas and the importance of correlating multiple imaging modalities when assessing atypical atrial masses. Our case highlights the potential role of multimodal imaging, including contrast echocardiography, cardiac MRI, and coronary angiography, in differentiating vascular from non-vascular cardiac tumors. Based on recurrent patterns in the literature, we outline a conceptual diagnostic framework that may assist preoperative evaluation. CONCLUSIONS: Although rare, cardiac hemangiomas should be considered in the differential diagnosis of atrial masses. Multimodal imaging may improve diagnostic accuracy, but additional data from multicenter registries are required to establish validated diagnostic pathways.