Cardiac tumors

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Cardiac Tumors - An Overview

  • Primary Tumors: Rare overall. Benign are more common than malignant.
    • Benign (most frequent primary type):
      • Adults: Atrial Myxoma. Typically in the left atrium. Can cause a "ball-valve" obstruction, leading to syncope or embolic strokes.
      • Children: Rhabdomyoma. Strongly associated with Tuberous Sclerosis.
    • Malignant: Angiosarcoma is the most common.
  • Metastatic Tumors: Spread from another primary site.
    • Common sources: Melanoma, lung cancer, breast cancer, and lymphoma.

⭐ Metastatic tumors are 20-40 times more common than primary cardiac tumors.

Atrial Myxoma Gross Specimen

Atrial Myxoma - The Classic Ball‑Valve

  • Most common primary cardiac tumor in adults; ~90% arise in the atria (LA > RA), often attached to the fossa ovalis.
  • Clinical Features:
    • "Ball-valve" obstruction: A pedunculated mass flops into the mitral valve during diastole, causing syncope, dyspnea, and mimicking mitral stenosis.
    • Constitutional symptoms: Fever, weight loss, and malaise due to cytokine production (esp. IL-6).
    • Auscultation: Characteristic early diastolic "tumor plop".
  • Histology: Composed of scattered stellate-shaped myxoma cells suspended in a gelatinous myxoid stroma (abundant mucopolysaccharide ground substance).
  • Complications: High risk of systemic embolization, leading to stroke or other infarcts.

Echocardiogram: Left atrial myxoma prolapsing mitral valve

⭐ Associated with Carney Complex, an autosomal dominant syndrome featuring multiple myxomas (cardiac, skin), spotty pigmentation (lentigines), and endocrine tumors.

Other Tumors - Rhabdomas & Rascals

  • Cardiac Rhabdomyoma
    • Benign hamartoma; the most common primary heart tumor in infants and children.
    • Location: Often multiple, favoring the ventricular walls and septum.
    • Clinical: May cause outflow obstruction, arrhythmias, or be asymptomatic.
    • Histo: Pathognomonic large, polygonal “spider cells” (glycogen-filled vacuoles with cytoplasmic strands).
    • Prognosis: Most spontaneously regress; surgery only if symptomatic.

⭐ Over 50% of cases are associated with Tuberous Sclerosis (TSC1/TSC2 gene mutations).

Cardiac Rhabdomyoma Histopathology with Spider Cells

Clinical Picture - Diagnosis & Effects

  • Presentation: Highly variable; depends on tumor size, location, and mobility.
    • Systemic/Constitutional: Fever, weight loss, malaise (cytokine release, esp. myxoma).
    • Obstructive: Mimics valve stenosis. Syncope, dyspnea, heart failure from mass effect.
    • Embolic: Tumor fragments break off → stroke, MI, limb ischemia.
    • Arrhythmias: Conduction system invasion → palpitations, heart block.

⭐ Left atrial myxomas classically cause a "ball-valve" obstruction, leading to syncope, and can present with stroke-like symptoms from systemic embolization.

High‑Yield Points - ⚡ Biggest Takeaways

  • Metastases are the most common cardiac tumors, far more than primary tumors.
  • Myxoma is the most common primary adult tumor, typically in the left atrium, causing "ball-valve" obstruction and constitutional symptoms (IL-6).
  • Rhabdomyoma is the most frequent primary pediatric tumor, strongly associated with tuberous sclerosis.
  • Angiosarcoma is the most common primary malignant cardiac tumor.
  • Common sources of metastases include melanoma, lung, and breast cancer.

Practice Questions: Cardiac tumors

Test your understanding with these related questions

A 48-year-old woman comes to the physician because of progressively worsening dyspnea on exertion and fatigue for the past 2 months. She had Hodgkin lymphoma as an adolescent, which was treated successfully with chemotherapy and radiation. Her father died from complications related to amyloidosis. She does not smoke or drink alcohol. Her temperature is 36.7°C (98°F), pulse is 124/min, respirations are 20/min, and blood pressure is 98/60 mm Hg. Cardiac examination shows no murmurs. Coarse crackles are heard at the lung bases bilaterally. An ECG shows an irregularly irregular rhythm with absent P waves. An x-ray of the chest shows globular enlargement of the cardiac shadow with prominent hila and bilateral fluffy infiltrates. Transthoracic echocardiography shows a dilated left ventricle with an ejection fraction of 40%. Which of the following is the most likely cause of this patient's condition?

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Flashcards: Cardiac tumors

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A mitral valve with diffuse fibrous thickening and distortion, commisural fusion at the leaflet edges, and narrowing of the mitral valve orifice is highly suggestive of what pathology?_____

TAP TO REVEAL ANSWER

A mitral valve with diffuse fibrous thickening and distortion, commisural fusion at the leaflet edges, and narrowing of the mitral valve orifice is highly suggestive of what pathology?_____

Mitral stenosis

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