A 60-year-old lady with neurofibromatosis presented with a long-standing history of paroxysmal palpitations, which had worsened recently. She had no risk factors for heart disease. Routine blood tests, thyroid function and tests to rule out pheochromocytoma were all normal. Holter monitoring showed asymptomatic paroxysmal atrial fibrillation. Chest X-Ray was reported as normal. Transthoracic echocardiography (parasternal long-axis view) (Figure 1) showed a large cyst-like mass posterior to the left atrium and left ventricle. Magnetic resonance imaging with intravenous contrast (Figure 2) showed all cm X 5 cm X 4 cm well-defined heterogeneously enhancing oval mass which extended from the level of the left atrium superiorly, to below the diaphragm at the level of the stomach inferiorly. Posteriorly the mass was abutting against the descending aorta and the esophagus was displaced laterally, towards the left. There was no intraspinal extension of the tumor. In a patient with neurofibromatosis with a posterior mediastinal and retroperitoneal mass, this was most likely to be a neural sheath tumor. The patient underwent uneventful excision of the mass through a left thoraco-laparotomy. Histology showed a benign schwannoma. Her palpitations resolved soon after the operation.
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