Cardiac tumors

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Classification - The Tumor Lineup

  • Secondary (Metastatic) > Primary
    • Metastases are 20-40x more common.
    • Sources: Melanoma, lung, breast, lymphoma.
  • Primary Tumors (Benign > Malignant)
    • Benign (~75%):
      • Myxoma: Most common in adults (left atrium).
      • Rhabdomyoma: Most common in children (tuberous sclerosis).
      • Fibroma, Lipoma.
    • Malignant (~25%):
      • Angiosarcoma: Most common primary malignant.

Cardiac Tumors: Location and Gross Appearance

⭐ Atrial myxomas can cause constitutional symptoms (fever, weight loss) via IL-6 production and a characteristic "tumor plop" on auscultation.

Atrial Myxoma - Jelly Ball Menace

Atrial Myxoma Histology (H&E)

  • Epidemiology: Most common primary cardiac tumor in adults. Benign, but clinically malignant due to effects.
  • Location: ~75% arise in the left atrium, often attached to the interatrial septum at the fossa ovalis.
  • Pathology: Gelatinous, friable mass ("jelly ball") composed of myxoma cells in a myxoid stroma.
  • Clinical Triad:
    • Constitutional: Fever, weight loss, malaise (due to IL-6 production).
    • Obstructive: Mimics mitral stenosis (dyspnea, syncope). Positional symptoms are common. Auscultation may reveal a diastolic "tumor plop."
    • Embolic: Fragments can break off, causing systemic emboli (e.g., stroke) or pulmonary emboli if right-sided.

Association: Multiple myxomas, often in younger patients, are associated with Carney Complex (autosomal dominant syndrome: myxomas, spotty pigmentation, endocrine tumors).

  • Rhabdomyoma

    • Most common primary cardiac tumor in children; often multiple.
    • Associated with Tuberous Sclerosis (TSC).
    • Typically found in the ventricles.
    • Histology: Large, vacuolated "spider cells" containing glycogen.
    • High rate of spontaneous regression. Histopathology of cardiac rhabdomyoma with spider cells
  • Angiosarcoma

    • Most common primary malignant cardiac tumor in adults.
    • Arises from vascular endothelium.
    • Usually originates in the right atrium.
    • Presents with right-sided heart failure, hemorrhagic pericardial effusion.
    • Poor prognosis due to early metastasis.

High-Yield: Over 50% of children with cardiac rhabdomyomas have Tuberous Sclerosis. Conversely, up to 80% of neonates with TSC will have a rhabdomyoma.

Metastatic Tumors - The Real Invaders

  • 20-40x more common than primary cardiac tumors. The heart is rarely the first site of metastasis.
  • Common Primaries: Lung & breast (direct extension), melanoma (hematogenous), lymphoma & leukemia (lymphatic/hematogenous).
    • 📌 Mnemonic: My Lungs Bleed Leukemia (Melanoma, Lung, Breast, Leukemia/Lymphoma).
  • Clinical Picture: Often asymptomatic. If symptomatic, usually due to pericardial involvement.
    • Presents as pericardial effusion, tamponade, or constrictive pericarditis.
    • Arrhythmias or heart block if myocardium is invaded.

⭐ Melanoma has the highest propensity for cardiac metastasis; up to 50% of patients with metastatic melanoma have cardiac involvement found at autopsy.

Echocardiogram: Pericardial effusion & cardiac mass

Diagnosis & Management - Find and Fix

  • Diagnosis:
    • Echocardiography (TTE/TEE) is the initial and primary diagnostic tool.
    • Cardiac MRI/CT for superior tissue characterization and surgical planning.
    • Biopsy is high-risk (embolization) and usually avoided pre-operatively.
  • Management:
    • Surgical resection is the definitive treatment for most primary tumors.
    • Benign tumors (e.g., myxoma) are often curative post-resection.
    • Malignant tumors may require adjuvant chemotherapy/radiation.

Echocardiogram: Left Atrial Myxoma Prolapsing Mitral Valve

⭐ Myxomas can present with constitutional symptoms (fever, weight loss) and elevated inflammatory markers (↑ESR, ↑CRP), mimicking endocarditis or vasculitis.

High‑Yield Points - ⚡ Biggest Takeaways

  • Metastatic tumors are far more common than primary ones, typically from lung, breast, or melanoma.
  • Most common primary tumor in adults: atrial myxoma, usually in the left atrium.
  • Myxomas present with constitutional symptoms, embolic events, or "ball-valve" obstruction.
  • Most common in children: rhabdomyoma, strongly associated with tuberous sclerosis.
  • Rhabdomyomas often regress spontaneously in children.
  • The most common malignant primary tumor is angiosarcoma.

Practice Questions: Cardiac tumors

Test your understanding with these related questions

A 68-year-old man comes to the emergency department because of a 1-week history of difficulty breathing. He has had recurrent palpitations over the past 2 years. During this time, he has also had several episodes of anxiety despite no change in his daily life. He has occasional sharp chest pain localized to the left upper sternal border. He has no abdominal pain or leg swelling. Two years ago, he had streptococcal pharyngitis, which was promptly treated with a 10-day course of penicillin. He has never traveled outside of the country. His temperature is 36.5°C (97.7°F), pulse is 82/min, and blood pressure is 140/85 mm Hg. Physical examination shows a 3/6 holosystolic murmur that is loudest at the apex and radiates to the axilla with a mid-systolic click. Bilateral fine crackles are heard on lung auscultation. Which of the following is the most likely cause of this patient's symptoms?

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Flashcards: Cardiac tumors

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One complication associated with lung cancer is _____, which can lead to obstructive shock

TAP TO REVEAL ANSWER

One complication associated with lung cancer is _____, which can lead to obstructive shock

pericardial effusion

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