A giant cardiac malignant peripheral nerve sheath tumor presenting with total obstruction of the superior vena cava.

Abstract

A 16-year-old boy presenting with dyspnea, facial swelling, cyanosis, and fatigue was found to have a tumor involving the heart, causing superior vena cava and brachiocephalic venous trunk total obstruction. This was diagnosed as malignant peripheral nerve sheath tumor, a rare sarcoma of the heart. The patient underwent successful resection of the tumor, and reconstruction of the superior vena cava and right atrium. Immunohistochemistry was utilized to establish the diagnosis. The details of the patient's clinical course and imaging findings with morphologic and immunohistochemistry data are reported.