Malignant peripheral nerve sheath tumor presenting as mitral stenosis: case report and literature review

Primary cardiac neurogenic tumors are rare, accounting for 5%-10% of all malignant soft tissue tumors. Among these, malignant peripheral nerve sheath tumors (MPNST) are particularly uncommon as primary cardiac tumors. This case involves a 55-year-old female with a history of type II diabetes mellitus and hypertension, who presented with dyspnea on exertion and episodes of presyncope. Initial evaluations, including chest X-ray, electrocardiography, and laboratory tests, showed no significant abnormalities. However, a transthoracic echocardiogram revealed a large mass attached to the mitral valve, which was prolapsing into the left ventricle. Further examination using transesophageal echocardiography showed two masses on both mitral leaflets. The patient underwent resection of the masses via median sternotomy, and histopathological analysis confirmed that the masses were MPNST. MPNST is an aggressive type of sarcoma arising from the cardiac branches of the vagus nerve. Diagnosis is typically made through echocardiography, computed tomography (CT), or cardiovascular magnetic resonance imaging (MRI). Early detection and prompt surgical resection remain the cornerstone of management in these rare tumors .