Case of an intracranial malignant peripheral nerve sheath tumor in the setting of pacer-dependent heart block

Abstract

Intracranial malignant peripheral nerve sheath tumors (MPNSTs) are an extremely rare entity with only a handful of cases reported in the literature. MPNSTs typically occur in the extremities and the trunk. The treatment algorithm includes, when possible, gross-total resection as these tumors are extremely aggressive. When these tumors occur intracranially, they are termed malignant intracerebral nerve sheath tumors. The diagnosis hinges on immunohistochemistry and pathological features and often the diagnosis can be delayed for this reason. In this setting, it is critical to utilize intraoperative navigation, thus necessitating the use of fine-cut magnetic resonance imaging (MRI). This report presents a patient who presented with symptoms of obstructive hydrocephalus secondary to an intracranial mass. The patient underwent a full and extensive metastatic workup that was ultimately negative. To complicate things, the patient was fully pacemaker dependent. In this report, we review the literature surrounding this type of tumor, along with a detailed presentation of the case mentioned including the difficulties of cardiac pacing in the setting of MRI.

Malignant Peripheral Nerve Sheath Tumor of the Cerebellar Hemisphere: An Unusual Location and Multiple Intracranial Parenchyma Metastases

“…Neurofibromatosis type-1 (NF1) patients have a cumulative lifetime risk of developing MPNST of 8-16%, and the predilection age is 20-35 years [2]. MPNST is more common in the trunk and extremities but rare in the brain, where it is also called malignant intracerebral nerve sheath tumor (MINST) [4]. Enrico Martin et al analyzed 3267 cases of MPNSTs of which 2329 cases (71.3%) were in the trunk and limbs, and 167 cases (5.1%) were in the cranium [5].…”

“…At present, it is believed that there is a correlation between the tumor and neurofibromatosis and radiation. Chica et al reported a 38-year-old MINST patient who received radiotherapy for Hodgkin's lymphoma during childhood [4]. Baehring et al found MPNST in three out of 54 patients, and these three patients had a history of radiotherapy [13].…”

The Utility of Magnetic Resonance Imaging-compatible Pacemakers in Neurosurgical Patients

“…A 38-year-old female [ 8 ] with a past medical history of Hodgkin lymphoma, treated with chemotherapy and radiation, along with radiation-induced heart conduction defect status post pacemaker placement, presented to a hospital in Mexico with a chief complaint of occipital headaches, dizziness, dysarthria, and imbalance. As a result, a CT scan was performed and demonstrated a midline superior cerebellar mass with surrounding edema, early hydrocephalus, and a small tentorial subdural hematoma (Figure 3 ).…”