Cardiac rhabdomyomas in childhood: six cases from a single institution

Area of Science:

• Pediatric Cardiology
• Pediatric Oncology
• Cardiovascular Pathology

Background:

• Primary cardiac tumors in children are uncommon.
• Rhabdomyomas are the most frequent primary cardiac tumors in pediatric populations.
• These tumors can present significant diagnostic and management challenges.

Purpose of the Study:

• To review the clinical characteristics, treatment outcomes, and follow-up results of pediatric patients diagnosed with primary cardiac rhabdomyomas.
• To evaluate the efficacy of different management strategies, including surgical resection and conservative follow-up.
• To identify factors influencing the prognosis and decision-making for symptomatic rhabdomyomas.

Main Methods:

• Retrospective review of six pediatric patients with primary cardiac rhabdomyomas.
• Diagnosis confirmed using magnetic resonance imaging (MRI) and echocardiography, with optional histopathology.
• Assessment of clinical presentation, tumor location, treatment interventions, and patient outcomes during follow-up.

Main Results:

• The mean age of patients was 16.8 days, with only one symptomatic individual.
• Tumors predominantly originated in the left ventricle.
• Two patients underwent complete tumor resection; significant tumor regression was observed in three patients post-intervention or spontaneously, with one stable tumor at a median follow-up of 39 months.

Conclusions:

• Primary cardiac rhabdomyomas in children often exhibit spontaneous regression, suggesting that close observation may be adequate for hemodynamically stable, asymptomatic cases.
• Despite potential for regression, rhabdomyomas can pose a risk of sudden death, necessitating further research into optimal surgical and follow-up strategies.
• Tumor localization and infiltrative nature are critical considerations for surgical decision-making in symptomatic pediatric rhabdomyomas.