Primary malignant peripheral nerve sheath tumour of the heart

Abstract

<jats:title>Abstract</jats:title><jats:p>A malignant peripheral nerve sheath tumour (MPNST) is a rare variety of soft-tissue sarcoma of ectomesenchymal origin. The World Health Organisation created the term MPNST to replace previous terminology such as malignant schwannoma, malignant neurilemmoma, neurogenic sarcoma, and myxofibrosarcoma for tumours of neurogenic origin with similar biological behavior.[1–3] The vast majority of these tumours develop in extremities. They also tend to be located in unusual sites of the body, such as the pelvic retroperitoneum, infratemporal fossa, intrapericardium, and mediastinum.[1,3,4] This case study presents a patient with an extremely rare primary cardiac MPNST..</jats:p>

A Rare Case of Left Ventricular Malignant Peripheral Nerve Sheath Tumour—Case Report and Review of the Literature

“…Most of the MPNSTs that affect the heart occur on the right side, close to the interatrial septum. The reason for this location might be the origin of the MPNST from the cardiac branches of the vagus nerve [ 8 , 9 , 10 , 11 ]. A few cases have been reported on the left side of the heart.…”

“…A few cases have been reported on the left side of the heart. About 50% of these tumours arise de novo, the other half arise from neurofibromas in the context of type 1 neurofibromatosis [ 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 ]. Patients with neurofibromatosis type I have an 8–13% greater risk of developing an MPNST than the general population [ 19 , 20 ].…”

Primary Malignant Peripheral Nerve Sheath Tumor of the Pulmonary Vein: A Case Report

“…예후는 불량하여 수술적 절제 후에도 높은 40%–60%의 재발률과 30%–60%의 전이율로 인해 중간 생존율은 3개월에서 1년으로 알려져 있다. 나쁜 예후를 시사하는 소견으로는 5 cm보다 큰 경우, 병리적으로 고등급인 경우, 수술 절제면에 침습이 있는 경우와 NF 1 형인 경우이다( 10 ). 본 증례의 경우 역시 크기가 5 cm 보다 컸으며 고등급이었다.…”

Primary Cardiac Schwannoma: A Meta-Analysis of Individual Case Reports

“…They are characterized by a higher likelihood of invading surrounding structures and metastasizing [22,24]. They are often symptomatic and can cause arrhythmias, embolic events, chest pain, and compression symptoms [17,[20][21][22][24][25][26]29,35,[43][44][45][46]48,49,51,52,54,[60][61][62][63]72]. In this meta-analysis, more than 50% of the reported cases were malignant (29/53 cases) which denotes that the incidence of cardiac MPNST is more than that of benign cases.…”