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Introduction

Atrial myxomas are the most common benign primary cardiac tumors, with approximately 80% located in the left atrium and the remaining arising from the right atrium or left ventricle.

Risk factors

  • Age:  Atrial myxomas are most common in young adults, with a peak incidence in the third and fourth decades of life.
  • Sex:  Women are slightly more likely to develop atrial myxomas than men.
  • Family history:  Family history of atrial myxoma or other cardiac tumors may increase the risk.
  • Genetic predisposition:  Certain genetic syndromes, such as Carney complex (autosomal dominant disorder with multiple tumors, including myxomas, schwannomas, and endocrine tumors), can be associated with an increased risk of atrial myxoma.

Pathology

The tumors are typically pedunculated, with a stalk arising from the atrial septum.  Histologically, these tumors demonstrate scattered myxoma cells within a mucopolysaccharide stroma and blood vessels, which may be encircled by myxoma cells (

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