A case report of primary cardiac intimal sarcoma presenting with atrial fibrillation and a left atrial mass

Abstract

Background: Intimal sarcoma is an exceedingly rare type of primary cardiac tumour. It is characterized by poorly differentiated spindle-shaped cells that can mimic smooth muscle and is strongly associated with MDM2 genetic amplification. Owing to its rarity and non-distinctive histological features, diagnosis remains a significant challenge. Case summary: In this case report, we describe a case of primary cardiac intimal sarcoma in a 37-year-old woman who presented with atrial fibrillation (AF) and a left atrial mass. Despite having a histological sample from an excised left atrial mass, the diagnosis was not made until she presented with back pain secondary to metastatic disease to the spine. Discussion: Primary cardiac intimal sarcoma is an extremely rare diagnosis. The mainstay management of intimal cardiac sarcoma is aggressive surgical resection. Unfortunately, the prognosis of cardiac sarcomas remains very poor, with a mean survival between 3 months and 1 year. This case of cardiac intimal sarcoma highlights the difficulty in establishing a diagnosis, particularly given the unusual presentation of AF.