Rhabdomyoma

Rhabdomyoma 

Updated: 10/23/2020

© Jun Wang, MD, PhD

General features
  • Most common pediatric primary tumor of the heart
  • Usually in patients less than 1 year of age
  • Sporadic cases may be associated with other congenital heart diseases
  • If cardiac, usually associated with tuberous sclerosis
  • Rhabdomyomatosis: Multiple, usually less than 1 mm each
  • Cardiac tumor may cause sudden cardiac death
  • May regress spontaneously
Clinical presentations
  • Most common location: ventricular myocardium
  • Symptoms associated with size, location and number of tumor: obstruction, arrhythmias, congestive heart failure
Key morphological features
Diagnostic approaches
  • Echocardiography, MRI
  • Biopsy/histological assessment
Differential diagnoses
  • Fibroma: May have calcification, dense fibroblast and collagen
  • Myxoma: Myxoid background
  • Teratoma: Usually pericardial, three germ line cells
  • Hemangioma: Histologic features
Genetic abnormalities
  • TSC1 and TSC2 if tuberous sclerosis associated
Treatment
  • Excision


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Contents

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