Abstract

Introduction: Precise diagnosis of a cardiac mass has crucial prognostic and therapeutic implications. Case Presentation: A 23-year-old woman presented to an outside hospital 6 days after delivery with headaches, chest pain and very high BP. A CT angiogram found no pulmonary emboli but identified a mass in the right atrium. Surgical removal was attempted but had to be aborted because of inability to get clear margins. Frozen section showed atypical spindle cells, suggestive of sarcoma. Patient was transferred to our hospital for further management. TTE (Figure) revealed a large solid mass almost entirely filling the right atrium and a small pericardial effusion. CMR showed a 7.2 cm hypervascular mass in the right atrium with possible invasion of the superior vena cava, suggestive of angiosarcoma. Additional tissue sections showed vascular spaces lined by benign endothelial cells positive for the endothelial markers ERG, CD31 and CD34 and infiltrating the underlying myocardium; proliferation rate was <1%. Based on these findings a diagnosis of primary cardiac hemangioma was made. Subsequent PET CT showed mildly FDG-avid cardiac mass and no metastases. Discussion: Cardiac masses are a diagnostic challenge. Identification hinges on clinical history, location and multimodality imaging but histopathology and immunohistochemistry remain the gold standard for a definitive diagnosis. Primary cardiac tumors are rare, found only in <0.05% of autopsies; 80% are benign and 20% malignant. Hemangiomas are extremely rare, representing <3% of primary cardiac tumors. They are often misdiagnosed as other neoplasms, as was the case in our patient, possibly leading to unnecessary invasive procedures. Although benign, hemangiomas can infiltrate surrounding structures and cause serious complications, such as syncope, emboli, arrhythmias, heart block and stroke. Complete surgical excision remains the treatment of choice and, if successful, confers a positive prognosis.

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