- most common primary cardiac tumor
- most cases are sporadic. 10% are familial (autosomal dominant)
- majority are solitary, usually develop in left atrium (75-85%) and are polypoid, gelatinous structures attached by a pedicle to atrial septum
- may obstruct mitral valve and can send showers of emboli
- constitutional symptoms in a third of cases
- signs: loud first heart sound, tumor 'plop' (loud third heart sound produced as pedunculated tumor comes to an abrupt halt), mid diastolic murmur and signs due to embolism
- raised ESR
- rarely, associated with adrenal hyperplasia, Leydig cell tumor and Carney complex
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