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An In Vivo Murine Sciatic Nerve Model of Perineural Invasion
Published on: April 23, 2018
The almost-invisible perineurioma
Carlos E Restrepo1, Kimberly K Amrami2, Benjamin M Howe2
1Departments of 1 Neurologic Surgery.
Abstract:
Intraneural perineurioma is a rare, benign slow-growing lesion arising from the perineurial cells that surrounds the peripheral nerve fibers. Typically it presents during childhood and young adulthood as a motor mononeuropathy. MRI plays an essential role in the diagnosis and localization of the lesion, which appears as a fusiform enlargement of the nerve fascicles that enhances intensely with gadolinium. Despite the typical clinical and radiological features, intraneural perineurioma remains largely underdiagnosed because of the lack of familiarity with this entity, but also as a result of technical limitations with conventional MRI that is typically performed as a screening test over a large field of view and without contrast sequences. The purpose of this article is to present the pitfalls and pearls learned from years of experience in the diagnosis and management of this relatively rare condition. Clinical suspicion and detailed neurological examination followed by high-quality electrophysiological studies (EPS) must lead to an adequate preimaging localization of the lesion and narrowing of the imaging area. The use of high-resolution (3-T) MRI combined with gadolinium administration will allow adequate visualization of the internal anatomy of the nerve and help in differentiating other causes of neuropathy. In cases where the lesion is not recognized but clinical suspicion is high, possible errors must be assessed, including the EPS localization, area of imaging, MRI resolution, and slice thickness.
Insights
Intraneural perineurioma, a rare nerve tumor, is often underdiagnosed. High-resolution MRI with gadolinium contrast is crucial for accurate diagnosis and management of this condition.
Area of Science:
- Neurology
- Radiology
- Oncology
Background:
- Intraneural perineurioma is a rare, benign tumor originating from perineurial cells, typically affecting peripheral nerves.
- It commonly presents as a motor mononeuropathy in children and young adults.
- Underdiagnosis is frequent due to unfamiliarity and limitations of conventional MRI.
Purpose of the Study:
- To present diagnostic and management strategies for intraneural perineurioma based on extensive experience.
- To highlight common pitfalls and offer pearls for improved diagnosis.
- To emphasize the importance of advanced imaging techniques.
Main Methods:
- Detailed neurological examination and high-quality electrophysiological studies (EPS) for lesion localization.
- High-resolution 3-Tesla (3-T) MRI with gadolinium contrast administration.
- Assessment of potential errors in EPS, imaging area, MRI resolution, and slice thickness.
Main Results:
- Intraneural perineurioma typically appears as a fusiform nerve enlargement with intense gadolinium enhancement on MRI.
- High-resolution MRI with contrast aids in visualizing nerve anatomy and differentiating from other neuropathies.
- Careful evaluation of diagnostic steps is essential when clinical suspicion is high but the lesion is not recognized.
Conclusions:
- Clinical suspicion, precise localization via EPS, and advanced MRI are key for diagnosing intraneural perineurioma.
- High-resolution MRI with gadolinium contrast is superior to conventional MRI for visualizing intraneural lesions.
- Addressing diagnostic pitfalls can improve the recognition and management of this rare nerve tumor.
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