Giant Mitral Valve Rhabdomyoma Resection in a Pediatric Patient
An 8-year-old girl weighing 20 kg was referred to the authors’
institution with progressive shortness of breath and fatigue. A
transthoracic and transesophageal echocardiogram revealed a 9 mm x 12 mm
tumor in the anterior and posterior leaflet of the mitral valve with
moderate to severe mitral regurgitation. Cardiac magnetic resonance
revealed a sessile and vascularized anterior and posterior mitral valve
tumor measuring 10 x 13 mm. After discussion among team members and
deterioration of clinical condition, surgical resection was decided.
Through a median sternotomy, cardiopulmonary bypass was instituted. After cardioplegic arrest, the left atrium was opened revealing a yellowish white lobulated giant cardiac tumor that compromised the totally of both the anterior and posterior mitral leaflets, with extension to the posteromedial papillary muscle precluding resection without valve damage. Therefore, mitral valve replacement was performed with a 25 mm mechanical mitral valve after tumor excision (Carbomedics Optiform, Sorin Group, Italy).
The postoperative course was uneventful and the patient was discharged after seven days. Macroscopic pathological examination revealed a smooth, lobulated, elastic, and yellowish white tumor measuring 5 x 3 x 1.5 cm. Microscopic examination demonstrated collagen fibrosis and benign mesenquimal neoplasia proliferation. The immunohistochemistry evidenced myoglobin and S100 positive compatible with mitral valve rhabdomyoma.
The authors present this case to report an unusual localization of the most frequent cardiac tumor in the pediatric population. Although different reports show the possibility of regression over the years, this could not happen and the patient could develop symptoms of heart failure. It is important to highlight that the possibility of clinical surveillance versus surgical resection, either partial or complete, could be limited to tumor size, location, and age of diagnosis.
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