Updated: 4/15/2020

Cardiac Tumors

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  • A 56-year-old man presents to his primary care physician for shortness of breath and episodes of lightheadedness. He says that his symptoms progressively worsened over the course of months. He recalls producing bloody sputum a few weeks ago and fainting for a few seconds yesterday night. Medical history is unremarkable. He denies alcohol, illicit drug, or cigarette use. Physical examination is remarkable for a "tumor plop" appreciated during diastole. An echocardiogram is performed and shows a left intra-atrial pedunculated mass. (Atrial myxoma)
  • Cardiac tumors
    • these can be divided into
      • primary cardiac tumors
        • myxoma (most common primary cardiac tumor)
          • occur mainly in the left atrium
        • rhabdomyoma
      • metastatic tumors
        • most common cardiac tumors overall
    • primary cardiac tumors are extremely rare and are more likely to be benign
Cardiac Tumors
Cardiac Tumors
  • Most commonly found in the atrium
    • preferably in the fossa ovalis of the atrial septum 
  • Presentation
    • Constitutional symptoms (fever, weight loss, and fatigue)
      • commonly due to IL-6 production by the myxoma
    • Can be friable and cause systemic embolization leading to stroke and other ischemic events
    • Diastolic "plop" may be auscultated
  • Masses can be pedunculated
    • this mass can make its way towards the atrioventricular valve opening during systole, resulting in an obstruction (which can lead to syncope) and damaging the valve leaflets 
      • echocardiography will show pedunculated mass
  • Histology
    • complex structures surrounding blood vessels and may appear as cords, rings, nests, or poorly formed glands
    • abundant mucopolysaccharide ground substance
      • consists of chondroitin sulfate and hyaluronic acid
    • consists of stellate or globular myxoma cells
      • eosinophilic cytoplasm
      • ill-defined cell borders
        • oval nucleus
          • indistinct nuclei
          • open chromatin
  • Most common primary heart tumor in adults
  • Echocardiography can identify the tumor
  • Surgery is the definitive treatment
  • Approximately 50% of cases are associated with tuberous sclerosis 
  • Histology
    • "spider cells"
    • hamartomatous growths
  • More common in pediatric patients and typically found within the first year of life
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