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.

Long Noncoding RNA DSCAM-AS1 Facilitates Proliferation and Migration of Hemangioma Endothelial Cells by Targeting miR-411-5p/TPD52 Axis

“…Hemangioma (HA) is a benign tumor commonly diagnosed in childhood, specifically in infants with low birth weight as well as premature infants. The etiology and pathogenesis of HAs have been investigated in recent years, but not completely clarified because of its complexity [ 1 , 2 ]. Females are the majority of HA patients, with a female: male rate of approximately 3 : 1, and HA generally occurs about 14 days after birth [ 3 , 4 ].…”

Cardiac hemangioma in a difficult anatomical location presented with ventricular tachycardia. A rare case report

“…To date, indications for surgery of cardiac hemangiomas are controversial. Although it is incidentally detected in the majority of cases, most authors advocate surgical removal due to its unpredictable long‐term behavior such as risk of sudden cardiac death, rupture, or embolism 2,3,6,7 . Surgical resection was preferred for our patient as he had presented with documented ventricular tachycardia.…”

“…Wang et al 6 presented a case of ventricular arrhythmia consisting bigeminies and premature trigeminies in a patient with endocardial cardiac hemangioma who underwent complete mass excision. Malakan Rad et al 7 reported a congenital right atrial hemangioma associated with prolonged QTc who underwent complete tumor removal. Similarly, our patient presented with documented VT which caused his symptoms.…”

Current status of diagnosis and treatment of primary benign cardiac tumors in children

“…The effusion or mass effect may cause extrinsic compression on the heart and cardiac tamponade. (ii) Cardiac Hemangioma (CH): CH accounts for 2-3% of the primary cardiac tumors with no significant sex predilection (46). The right atrium is the most common location for fetal and neonatal CH, whereas the left ventricle is the most common site for CH in the adulthood (46,47).…”