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Published on: September 13, 2019
New-onset cardiac rhabdomyoma beyond infancy in a patient with tuberous sclerosis complex
Nikhil M Thatte1, Kristine J Guleserian2, Surendranath R Veeram Reddy3
11Department of Pediatrics,University of Texas Southwestern Medical Center,Dallas,Texas,United States of America.
Insights
New cardiac rhabdomyomas (tumors) can develop after infancy in children with tuberous sclerosis complex. This rare occurrence in a young boy highlights the need for ongoing heart monitoring in these patients.
Area of Science:
- Cardiology
- Pediatric Oncology
- Genetics
Background:
- Tuberous sclerosis complex (TSC) is a genetic disorder.
- Cardiac rhabdomyomas are common in TSC, typically appearing in infancy.
- These tumors often stabilize or regress spontaneously.
Observation:
- A male infant with TSC required surgical removal of a cardiac rhabdomyoma.
- At two years of age, a new rhabdomyoma developed in a different cardiac location.
- This new tumor also necessitated surgical resection.
Findings:
- The development of new cardiac rhabdomyomas beyond infancy in TSC patients is exceptionally rare.
- This case demonstrates the potential for late-onset rhabdomyoma formation in TSC.
Implications:
- Routine cardiac surveillance is crucial for all patients with tuberous sclerosis complex, even if asymptomatic.
- Continuous monitoring may be necessary to detect new or recurrent cardiac rhabdomyomas.
- This case underscores the importance of long-term follow-up in managing TSC-related cardiac manifestations.
Abstract:
Cardiac rhabdomyomas in patients with tuberous sclerosis complex are usually detected antenatally or during infancy, with subsequent stabilisation or spontaneous regression. Development of a new cardiac rhabdomyoma beyond infancy is very rare. We report a male child who needed resection of a large rhabdomyoma in neonatal life, and then developed a new-onset rhabdomyoma at 2 years of age in a different location, needing another resection. Routine surveillance for cardiac rhabdomyomas in asymptomatic patients with tuberous sclerosis is essential.
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