cardiac rhabdomyoma
Benign cardiac tumor composed of striated muscle cells, most commonly seen in infants and strongly associated with tuberous sclerosis.
Full Definition
Cardiac rhabdomyoma represents the most common primary cardiac tumor in infants and children, consisting of benign proliferations of striated muscle cells within the myocardium. These tumors are strongly associated with tuberous sclerosis complex, with up to 80% of patients having this genetic disorder. Histologically, rhabdomyomas are characterized by large cells with abundant eosinophilic cytoplasm, clear vacuoles, and centrally located nuclei, creating the characteristic 'spider cell' appearance. Multiple tumors are common, and they may regress spontaneously over time. The tumors can cause arrhythmias, outflow tract obstruction, or other hemodynamic compromise depending on their size and location.
Usage
Usage note: Always distinguish from rhabdomyosarcoma, which is malignant and has different morphology.
In Context
- "Multiple cardiac rhabdomyomas were identified in the ventricular septum and free wall of both ventricles." — Pediatric cardiac pathology report
- "The presence of cardiac rhabdomyoma prompted genetic testing for tuberous sclerosis complex." — Pediatric cardiology consultation