Abstract
Primary cardiac tumors are rare, and myxomas represent the most common type. Myxoma in a child is very rare. Herein, we present the case of a 4-year-old boy who was diagnosed with a right atrial mass two months after open-heart surgery for removal of a supra-mitral ring and repair of an interatrial septum aneurysm. He underwent surgery for resection of the mass, and pathological studies showed it was a cardiac myxoma. After 45 months of follow-up, the patient was alive without any recurrence or problems. This is the most rapidly growing myxoma ever reported in a child.
Introduction
Primary cardiac tumors are rare with estimated rates of 0.001% to 0.28% according to postmortem studies. 1 Nearly 70% of primary cardiac tumors are benign, and myxomas account for half of these benign tumors. 2 The left atrium is the most common site of cardiac myxomas, followed by the right atrium. 3 Sporadic reports have attempted to define the growth rate of atrial myxomas, and although there is little information regarding the period in which a myxoma might be present before diagnosis, they are considered to be slow-growing tumors. 4 However, rapid growth of atrial myxomas has been reported in unusual cases in both children and adults.4,5 We present the case of a 4-year-old boy who developed a de-novo right atrial myxoma two months after cardiac surgery for removal of a supra-mitral ring and repair of an interatrial septum aneurysm.
Case report
A 4-year-old boy was referred to our hospital with the diagnosis of a right atrial mass. He had undergone two previous operations: repair of coarctation of the aorta through a left posterolateral thoracotomy; and 3 years later, open-heart surgery for resection of a supra-mitral ring and repair of an interatrial septum aneurysm that was resected, and the atrial septal defect was repaired by direct closure. Postoperative examination by transthoracic echocardiography (TTE) showed excellent results without any residual defect. Two months later during routine follow-up, a right atrial mass was detected by TTE. The patient was asymptomatic. TTE revealed a pedunculated clearly delineated mass measuring 18 × 9 mm, adjoining the inferior vena cava (Figure 1). The patient was admitted and scheduled for urgent surgery. The operation was performed using cardiopulmonary bypass without arresting the heart. The right atrium was opened, and a 1 × 2-cm mass was seen. The mass was attached to the lateral wall of the right atrium, prolapsing across the tricuspid valve. The mass was excised from the right atrial wall, along with its base. The right atriotomy was closed, and the patient was weaned off bypass. He had an uneventful recovery period in the intensive care unit. TTE confirmed complete removal of the mass without any defects. Pathological study of the mass revealed polygonal round or spindle-shaped cells (myxomatous cells) showing mild cellular atypia and separated by a myxomatous matrix containing arborizing blood vessels; the diagnosis was myxoma of the heart. The patient was discharged from the hospital on the fifth postoperative day. He was followed-up for 45 months, having a normal life without any recurrence of the mass or any other problems.
Echocardiographic apical short-axis view showing the mass in the right atrium, adjoining the inferior vena cava.
Discussion
The incidence of myxoma in children is very rare, and most reported cases have been in patients with normal hearts or with isolated atrial septal defects, with one case of right atrial myxoma in an unoperated child with double-outlet right ventricle. 5 Generally, atrial myxomas present with obstructive symptoms (dyspnea, syncope), embolic events, constitutional symptoms (fever, arthralgia, myalgia, fatigue, rashes, and weight loss), or they may be silent.2,6,7 Our patient was a 4-year-old boy who was asymptomatic, and the right atrial mass was detected by TTE as part of routine follow-up after he had undergone open-heart surgery for repair of a supra-mitral ring and interatrial septum aneurysm. The mass was resected and pathological studies established the diagnosis of myxoma. Recurrence of myxoma is rare with an estimated rate of nearly 2%.8,9 After 45 months of follow-up of our patient, there was no evidence of recurrence. There are reported cases of rapidly growing myxomas in both in adults, 4 and children; 5 however, we think the tumor in our patient is the most rapidly growing myxoma ever reported in a child.
Footnotes
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.