Cardiac tumors presenting as mediastinal masses in childhood are rare. This report describes the case of a 6-year-old girl who presented to us after a failed attempt at resection of a mass arising from the left atrial appendage. Computed tomography-guided biopsy suggested a perivascular epithelioid cell tumor (PEComa). Sirolimus was started in order to reduce the mass size. She subsequently underwent resection of the mass by midline sternotomy approach and cardiopulmonary bypass. Recovery was uneventful and the child is doing well at two-year follow-up. Contemporary literature is reviewed. Only seven cases, including two children, with mediastinal PEComa have been reported. Pathology and treatment options are discussed.
Keywords: PEComa; arrhythmia; cardiac tumor; mediastinal tumor; sirolimus.