Unlike benign primary cardiac tumors like rhabdomyomas and fibromas, primary malignant tumors are extremely rare in children with very few cases reports in the literature so far. Rhabdomyosarcoma is a rare primary malignant tumor in children with most reported cases occurring in the right heart. We report a 15-month-old child with a primary rhabdomyosarcoma arising from the left ventricle and presenting with cardiac tamponade and circulatory failure. He underwent a diagnostic sternotomy and palliative chemotherapy but succumb to the disease later.
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