Malignant peripheral nerve sheath tumor of the heart: Case report

Abstract

The incidence of primary cardiac tumors ranges from 0.001% to 0.03%. Among the primary cardiac tumors, Malignant Peripheral Nerve Sheath Tumor (MPNST) of the heart is exceedingly rare: only few cases, according to our knowledge, are described in the literature up to now. The aim of this article was to present rare case of the MPNST and show the importance of the Magnetic Resonance Imaging in diagnosing and treating this rare tumor. We report the case of 43-year-old women presented with dyspnoea, chest pain, general malaise and arrhythmia. Cardiac magnetic resonance (CMR) imaging with a 1.5T Siemens Avanto scanner was performed and revealed a tumor in the left atrium. To capture the dynamics of the disease, two repeated follow-up studies were performed. The rare diagnosis - Malignant Peripheral Nerve Sheath Tumor (MPNST) of the heart - was established. In this case report we describe the clinical features of the heart MPNST and discuss the role of MRI in detection, staging, follow-up and therapy planning of this rare heart disease. In our case combination of surgery and radiotherapy was effective in treatment of MPNST with marked clinical improvement, although tumor did not disappear completely. Multiplanar CMR imaging is an advanced tool in diagnosis, treatment planning and follow-up of rare cardiac tumors.