Elsevier

Radiology Case Reports

Volume 18, Issue 2, February 2023, Pages 620-623
Radiology Case Reports

Case Report
CT of malignant peripheral nerve sheath tumor

https://doi.org/10.1016/j.radcr.2022.10.104Get rights and content
Under a Creative Commons license
open access

Abstract

Malignant peripheral nerve sheath tumors are soft tissue sarcomas that typically arise from a neurofibroma. Patients with neurofibromatosis type 1 represent approximately half of the population diagnosed with these tumors. This autosomal-dominant genetic disorder is distinguished by loss-of-function mutations in the neurofibromin 1 gene, which ultimately promotes atypical cellular proliferation. These biologically aggressive tumors are associated with a poor prognosis as they are resistant to available therapies and have high rates of recurrence, progression, and mortality. In this article, we report the case of a 45-year-old male with a history of neurofibromatosis type 1 who was diagnosed with a malignant peripheral nerve sheath tumor. We focus on optimizing diagnosis and treatment through the application of radiological imaging modalities, including cinematic rendering.

Keywords

Malignant peripheral nerve sheath tumor
Neurofibromatosis type 1
Neurofibromin 1 gene
Computed tomography

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Funding: There was no funding associated with this work.

Competing Interests: None of the authors have any conflict of interest to disclose.

1

All authors contributed equally to the writing of this manuscript.