367 LEFT VENTRICULAR MALIGNANT PERIPHERAL NERVE SHEATH TUMOR, A RARE PRIMARY CARDIAC NEOPLASM

Abstract

Abstract This is a case of a 71 year-old man, symptomatic for heart failure (NYHA II class), with recent weight loss and occurrence of arthralgia. In the last month episodes of non sustained ventricular tachycardia were observed. Transthoracic echocardiography showed a dilated left ventricle with reduced ejection fraction (30%) and the presence of an apical left ventricular cyst (dimensions 33×36 mm), with no doppler signal inside. No relevant valvular diseases were found. The first hypothesis was a cystic echinococcosis with left ventricle localization, but the serological testing resulted negative. In order to obtain a better characterization of the apical lesion a Cardiac Magnetic Resonance (CMR) was performed: it confirmed the apical mass with thin wall, high T2 and T1 signal and no contrast enhancement. CMR findings raised the suspicion of cardiac tumor. The case was discussed in the local heart team, and the patient was scheduled for cardiac surgery with mass removal. The histological examination showed a malignant mesenchymal neoplasm related to a primary cardiac schwannoma. Follow up outpatient visit showed a decrease of the arrhythmic burden and a progressive improvement in patient's symptoms. Echocardiography reported little improvement in EF (38%) and no suspicious lesions. MPNST (malignant peripheral nerve sheath tumor) is a type of cancer extremely rare and not fully considered in literature with a poor prognosis. CMR and echocardiography could help to early diagnose this neoplasms when dimension and localization are not prohibitive for operating.