Myxoma Cordis with Unusual Features

Cardiac myxoma is the most common primary tumor of the heart.¹ It mostly arises from the interatrial septum and involves the left atrium.² Herein, we report a case of unusual cardiac myxoma in the right ventricle.

A 25-year-old pregnant female (at the 10th gestational week) presented to our clinic with symptoms of dyspnea and palpitation. She underwent surgical excision of the parotid gland 10 years earlier with a final diagnosis of acinic cell carcinoma. On physical examination, she had signs of systemic venous congestion and mild tachycardia. A transthoracic echocardiogram revealed a giant mass in the right ventricle (measuring 41 by 15 mm), possibly originating from the apical region of the interventricular septum along with mild dilatation of right heart chambers and moderate tricuspid regurgitation (Figure 1A and Video 1). Following the termination of her pregnancy (based on a multi-disciplinary consensus), cardiac magnetic resonance imaging demonstrated findingspotentially suggestive of a primary malignant tumor (areas of necrosis and contrast enhancement, etc.) (Figure 1B and 1C). On the other hand, she had no signs of metastasis and no existing cancer susceptibility genes (as analyzed by next-generation sequencing method in DNA samples obtained from the patient's peripheral blood). Thereafter, surgical removal of the mass was performed. The histopathological examination was consistent with a cardiac myxoma (Figure 1D). She had an uneventful postoperative course and clinical follow-up. The present report potentially suggests a couple of implications: First, cardiac myxomas might also arise in atypical locations including the right ventricle. Second, even sophisticated imaging modalities might be inconclusive for the definitive diagnosis of cardiac tumors. Finally, certain physiological conditions including pregnancy might speed up the diagnosis of these tumors largely due to exacerbation of symptoms.

Figure 1.

(A) Right ventricular mass view from the apical 4-chamber window in transthoracic echocardiography. (B) Short-axis-balanced steady-state free precession sequence image demonstrates an irregular mass in the right ventricle. (C) Heterogeneous contrast enhancement is seen in right ventricle mass on the late gadolinium enhancement. (D) Histopathological appearance of myxoma.