Biventricular Myxomas

Sep 26, 2018   |  Grecia Iveth Maryelis Raymundo Martinez, MD; Laura Rodríguez-Chávez, MSc; Rodrigo Gopar-Nieto, MD
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A 20-year-old female patient had a 3-month history of progressive dyspnea. Physical examination showed isolated lentigines of the lower lip, palms of both hands, and left flank. Cardiovascular findings revealed a holosystolic heart murmur at the lower left sterna border. The electrocardiogram was in sinus rhythm with an incomplete right bundle branch block (Figure 1). Transesophageal echocardiogram revealed a right ventricular (RV) hypodense mass of 80 x 57 mm extending through all the RV toward the tricuspid valve with RV outflow tract obstruction and a smaller left ventricular (LV) apical mass of 10 x 10 mm. Severe mitral and tricuspid regurgitation were noted. The initial N-terminal pro b-type natriuretic peptide was 1,465 pg/ml. A contrast computed tomography scan was made, reporting a 22 HU mass in the RV of 50 x 48 x 71 mm that extended to the tricuspid valve; in the apical segment of the LV there was a similar mass of 14 x 15.5 x 12 mm (Figures 2-3).

Figure 1

Figure 1

Figure 2

Figure 2

Figure 3

Figure 3

The patient was taken to elective surgery, and the masses were resected and both mitral and tricuspid valves were replaced with bioprostheses (Figure 4). Histopathology confirmed a fragmented myxoma with a hyalinized pedicle without residual endocardial lesions (Figure 5). The complete hormonal panel was normal. Her family was studied. Both of her siblings had ephelides and lentigines; however, there was no evidence of cardiac tumors in any of her relatives. In 2016, the patient had a normal pregnancy with a cesarean section and no complications. Eight months later, a transthoracic echocardiography study showed no residual masses. During her follow-up in 2017, nodular lesions were found in her left breast. A breast ultrasound was revealed multiple nodules, with characteristics of myxomas versus myxoid fibroadenomas.

Figure 4

Figure 4
Macroscopic view of the tumors shows brown reddish color with myxoid and hemorrhagic areas. Left mass corresponds to the myxoma resected from the RV and right mass from the LV.

Figure 5

Figure 5
(A) Hematoxylin-eosin stains 10x microphotography. The tumor is formed of fusiform isolated cells with some cluster of fusiform cells. (B) Vimentin, mesenchymal cells stain in color brown. (C) Anti-Actin specific for smooth muscle staining the wall of the vessels. There is no evidence of malignant cells nor a smooth muscle origin. (D) Desmin stain, negative for malignant cells or striated muscle origin.

What is this patient's most likely diagnosis?

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