tumor

Large Asymptomatic Cardiac Tumor

Pankajkumar Shah, MD, Rao Velaga, MD,
and Rinkesh Patel, MBBS

A 69-year-old Caucasian male with known history of hypertension, peripheral vascular disease, and smoking presented to the surgeon with a neck mass. Prior to removal of the mass, he was referred for preoperative cardiac evaluation. 

Physical examination. On initial evaluation, he was in good health and denied chest pain, palpitation, shortness of breath, or syncope. He was afebrile with regular pulse. Cardiac examination did not find any abnormality. Although asymptomatic, we decided to perform further cardiac evaluation due to his significant risk factors. 

Laboratory tests. The electrocardiogram showed normal sinus rhythm without any abnormality. Subsequently, a transthoracic echocardiogram was done that revealed an enlarged left atrium measuring 5 cm and a sessile mass of 2 cm Ă— 3 cm in size, arising from the interatrial septum (Figure). 

Figure. Transthoracic echocardiograph.

Treatment. The patient was referred to the cardiothoracic surgeon for resection of the mass. On follow-up, the patient underwent uneventful surgical removal of the mass. The histopathological exam showed myxomatous changes in the tumor suggestive of myxoma.

Discussion. Primary cardiac tumors are very rare with an incidence of 0.02% to 0.1%.1,2 A majority (75%) of these tumors are benign, and nearly 50% of these benign cardiac tumors are myxomas.3 Other common benign tumors include lipomas, papillary fibroelastomas, and rhabdomyomas.

Cardiac myxomas occur as sporadic or as autosomal dominant syndrome carney complex.4 They are most commonly found in the left atrium, arising from the endocardium of interatrial septum, and presenting predominantly in adult women. Histologically, these tumors consist of stellate cells embedded in type IV collagen and loose connective tissue infiltrated with basophils.

Clinical presentation of cardiac myxoma includes signs and symptoms related to intracardiac obstruction, embolic manifestations, or constitutional symptoms—depending on its size and location.6 In about 4% of cases, it can be found on routine evaluation, and patients remain completely asymptomatic despite the large size, as seen in our patient.

Electrocardiogram and chest radiography can be suggestive of increase left atrial size. The gold standard diagnostic test is echocardiography, which can reveal the site of attachment, dimensions, other masses, and can measure any obstruction to outflow.8 Transthoracic echocardiogram is simple, noninvasive, and the preferred initial test. Transesophageal echocardiogram is more informative than transthoracic echocardiogram. Surgical removal is necessary due to the significant risk of sudden cardiac death and various embolic complications. 

References:

1. Lam KY, Dickens P, Chan ACL. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med. 1993;
117(10):1027-1031.

2. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996;
77(1):107.

3. Reynen K. Cardiac myxomas. N Engl J Med. 1995;333(24):1610-1617. 

4. Engberding R, Daniel WG, Erbel R, et al. Diagnosis of heart tumors by transesophageal echocardiography: a multicenter study in 154 patients. Eur Heart J. 1993;14:1223.

5. Krikler DM, Rode J, Davies MJ, et al. Atrial myxoma: a tumour in search of its origins. Br Heart J. 1992;67(1):89-91.

6. Stratakis CA, Kirschner LS, Carney JA. Clinical and molecular features of the carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab. 2001;86:4041-4046.

7. Meng Q, Lai H, Lima J, et al. Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases. Int J Cardiol. 2002;
84(1):69-75.

8.Sugeng L, Lang RM. Atypical cardiac myxomas. Echocardiography. 2004;
21(1):43-47.