Giant congenital right atrial epithelioid-capillary hemangioma with prolonged QT interval: Case report and practical surgical treatment strategy for primary cardiac tumors in children based on 25-year review of 299 cases.

Abstract

Cardiac hemangioma is very rare and accounts for 2%-3% of the primary cardiac tumors. Cardiac epitheloid-capillary hemangioma has not been reported in the pediatric population so far. We report the fatal outcome of a preterm neonate with a huge congenital right atrial epitheloid-capillary hemangioma and elevated serum alpha-fetoprotein, associated with prolonged QT interval. We describe the echocardiographic, computed tomographic (CT) imaging, microscopic and immunohistochemical features of the tumor. Complete resection of the tumor was done at operation necessitating extensive reconstruction of atrial walls. Intramural infiltration of this tumor into the surrounding myocardial walls is a challenging characteristic of cardiac hemangioma. The temptation to complete resection should be avoided in the setting of extensive intramural infiltration and entrapment of the tumoral cells into atrial walls, particularly in a preterm neonate. None of the current classifications for hemangioma was inclusive of our case. Based on a concise literature review of nine published classification systems from 1996 to 2017 , we discuss the shortcomings of the current classifications for hemangioma. We also performed a 25-year-review of 299 cases of primary cardiac tumors in neonates and children, from 1993 to May 2018. We suggest a stepwise surgical treatment strategy according to the characteristics of the patient and of the tumor, based on this review. The stepwise strategy includes watchful observation, partial resection, complete resection and cardiac transplantation.