In children, what primary cardiac tumor is often associated with tuberous sclerosis?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

The primary cardiac tumor that is frequently associated with tuberous sclerosis in children is a rhabdomyoma. Rhabdomyomas are benign tumors of striated muscle, and they are notably seen in the context of tuberous sclerosis, a genetic disorder characterized by the formation of benign tumors in multiple organs, including the heart, skin, and brain.

In tuberous sclerosis, the presence of rhabdomyomas can be a significant diagnostic criterion. These tumors often occur in the ventricles or myocardium and can lead to complications such as obstruction of blood flow, arrhythmias, and heart failure, particularly if they are numerous or large. The association between rhabdomyomas and tuberous sclerosis is so strong that the presence of rhabdomyomas in a child raises suspicion for this genetic disorder, influencing both diagnosis and surveillance for other potential manifestations of the condition.

Other tumors such as hemangiomas, fibromas, and myxomas may occur in children or in the general population but are not characteristically linked to tuberous sclerosis. This distinction helps reinforce the recognition of rhabdomyomas as a key feature in the clinical context of tuberous sclerosis, contributing to the understanding and

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