Epidemiology


  • Most common primary cardiac tumor in adults.
  • ~90% are sporadic.
  • Familial form: Carney complex (Autosomal Dominant; characterized by cardiac/extracardiac myxomas, spotty skin pigmentation/lentigines, and endocrine tumors).
  • ~80% locate in the Left Atrium (LA).

Etiology


Pathophysiology


Clinical features


  • Constitutional: Fever, weight loss, fatigue, Raynaud phenomenon (tumor secretes IL-6). c
  • Embolic: Stroke/TIA, acute limb ischemia, mesenteric ischemia, retinal artery occlusion (due to tumor fragmentation). c
  • Obstructive: Positional dyspnea, orthopnea, or syncope (often worse when upright, improves when supine due to mass obstructing the mitral valve). Mimics Mitral Stenosis (MS).
  • Physical Exam: Characteristic “tumor plop” (early diastolic sound immediately after S2), mid-diastolic rumbling murmur at the apex.
  • Complications
    • Sudden cardiac death (due to “ball-valve” mechanical obstruction of the mitral orifice).
    • Irreversible neurological deficits from cerebral emboli.
    • Arrhythmias (heart block or AFib).
    • Recurrence (1-3% in sporadic, up to 12-20% in familial/Carney complex).

Diagnostics


  • Initial: Transthoracic Echocardiogram (TTE) - reveals a mobile, pedunculated mass in the LA, classically attached to the interatrial septum at the fossa ovalis.
  • Confirmatory/Best: Transesophageal Echocardiogram (TEE) for detailed pre-op characterization, followed by definitive Histopathology post-resection (scattered spindle cells within a mucopolysaccharide stroma).
  • Key Labs: ↑ ESR/CRP, normochromic normocytic anemia, thrombocytosis (all acute-phase reactant responses driven by IL-6).

Treatment