Key result
Primary cardiac intimal sarcoma presents unusually with AF and an LA mass before spinal metastasis.
Why the study?
Primary cardiac intimal sarcoma is exceedingly rare with non-distinctive histological features, making diagnosis a significant clinical challenge.
Case Report (n=1)
Primary cardiac intimal sarcoma is an extremely rare and difficult-to-diagnose tumor that can present unusually with atrial fibrillation.
Consider malignancy in young patients with new AF and left atrial mass; extends reported presentations of cardiac intimal sarcoma.
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.
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Ho et al. (2021) conducted a case report in Primary cardiac intimal sarcoma (n=1). A 37-year-old woman with primary cardiac intimal sarcoma presented unusually with atrial fibrillation and a left atrial mass, with diagnosis delayed until metastasis to the spine.
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