Unusually Early Recurrence of Mitral Valve Myxoma in a Child

A 16-year-old boy presented with fever, myalgia, and pain and swellings of fingers and toes. Osler's nodes and Janeway lesions were found on the palms and soles (Figure 1). Laboratory findings showed a leukocytosis and high C-reactive protein, but a negative blood culture. Echocardiography showed a huge, irregular shaped, mobile mass in the left ventricular outflow track (LVOT), attached to the anterior leaflet of mitral valve (MV) and protruded into LVOT during systole (Figure 2, and Supplementary Videos 1, 2, 3, 4). Brain magnetic resonance imaging and abdominal computed tomography showed multiple embolic infarctions in the brain, spleen, and kidneys (Figure 3). Immediate surgical excision with MV preservation was performed (Supplementary Video 5), showing a large, friable, and polypoid mass (Figure 4A). Histology confirmed MV myxoma (Figure 5). Fever subsided on the next day. The patient was discharged home 9 days after operation. One month later, fever, and hands and feet pains redeveloped. Echocardiography showed a recurrent myxoma at the anterior leaflet of MV (Figure 6, and Supplementary Videos 6 and 7). New acute embolic infarctions in the brain and spleen were also found. MV replacement was done after en bloc excision of the tumor and MV anterior leaflet (Figure 4B).

Figure 1
The patient had swellings of fingers and toes, and painful, red, and raised lesions (Osler's nodes), and non-tender, small erythematous or hemorrhagic macular lesions (Janeway lesions) on the palms and soles.

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Figure 2
Echocardiography showing a huge, irregular shaped, and heterogeneous mass attached to the anterior leaflet of mitral valve at an apical 4-chamber (A), a parasternal long-axis (B), and a parasternal short-axis (C) views.
LA = left atrium; LV = left ventricle.

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Figure 3
Brain magnetic resonance imaging and abdominal computed tomography showing multiple embolic infarct lesions (white arrows) in the brain (A), kidney (B), and spleen (C).
K = right kidney; S = spleen.

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Figure 4
(A) A gelatinous and polypoid mass excised from mitral valve at the first operation. (B) An en bloc mass with the recurred tumor and mitral valve tissues excised at the second operation.

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Figure 5
Histology of the mitral valve myxoma showing a lobulated, irregular, and frond-like surfaces, which is prone to embolization (A), and an abundant myxoid stroma with single spindle and stellate cells (B).

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Figure 6
At a month after the first operation, a new tumor was identified at the ventricular side of mitral valve at the apical 4-chamber (A) and parasternal long-axis (B) views.
LA = left atrium; LV = left ventricle.

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Most cardiac myxoma arises from the left atrium, while MV myxomas are very rare and mostly from atrial side of MV.1), 2) As our case, a huge myxoma originated from ventricular side of anterior MV has not been reported in children.3) In addition, tumor recurrence within a month is very unusual, which usually takes more than several months or years.4)

The Institutional Review Board of Seoul Saint Mary's Hospital approved this study and the patient's informed consent was waived (IRB number: KC21ZISI0322).