Vascular Tumors of the Heart in Infants and Children: Case Series and Review of the Literature

Primary tumors of the heart are rare in children, of which vascular tumors comprise a small subgroup. We present the clinical, histopathologic, and imaging findings in six children with vascular tumors of the heart and review the findings of 36 previously published cases. We observed three intramuscular hemangiomas of the small-vessel type in older children, two congenital hemangiomas in infants, and one malignant polymorphous hemangioendothelioma. Intramuscular hemangiomas did not respond to corticosteroid and were biologically distinct from the congenital hemangiomas, both of which exhibited regression with pharmacotherapy. Age at diagnosis appears to predict histologic type, tumor location, and clinical presentation.