Cardiac malignant peripheral nerve sheath tumours arising from atrial neurofibroma as an unusual complication of neurofibromatosis

Abstract

Currently, we report the case of a 60-year-old female patient, so far untreated cardiologically, admitted to the Clinic of Cardiology due to dyspnea, weakness and significant reduction of exercise tolerance. Echocardiography revealed the presence of a tumor in the left atrium, growing into the interatrial septum and penetrating into the right atrium. The tumor obstructed blood flow to the left ventricle and penetrated into the left ventricle during diastole. The magnetic resonance imaging (MRI) confirmed the presence of the tumor. Due to worsening of heart failure an urgent cardiac surgery was performed. Intraoperative examination revealed a large tumor measuring 8x6x4.5cm in the left atrium and 3.5x2x1.5cm in the right atrium that was blocking the mouths of pulmonary veins and the mitral valve and was invading the surrounding coronary sinus. The majority of tumor mass was excised with slender or doubtful surgical margins due the obvious reason following from location. Histopathological examination revealed a low-grade myxoid malignant peripheral nerve sheath tumor which developed from preexisting neurofibroma. After surgery, heart failure symptoms have been disappearing gradually. The patient has been under cardiological observation by standard MRI and also positron emission tomography. Disease free period was restricted to two years, than local recurrence has been stated. Secondary surgery has been performed and pathology report confirmed MPNST recurrence. To present day any distant metastases has not been revealed.