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

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Eur Heart J Case Rep. 2021 Oct 7;5(11):ytab410. doi: 10.1093/ehjcr/ytab410. eCollection 2021 Nov.


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.

PMID:34859182 | PMC:PMC8633751 | DOI:10.1093/ehjcr/ytab410