Modern treatment of perineuriomas: a case-series and systematic review

Anne-Kathrin Uerschels,Ulrich Sure,Karsten H Wrede,Oliver Gembruch,Christos Krogias,Andreas Junker

doi:10.1186/s12883-020-01637-z

Anne-Kathrin Uerschels, Ulrich Sure + Show 4 more

Open Access

https://doi.org/10.1186/s12883-020-01637-z

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Abstract

BackgroundPerineuriomas are rare benign peripheral nerve sheath tumours of perineurial cell origin and can be classified into intraneural and extraneural perineuriomas. They most commonly present a mononeuropathy of gradual onset and slow progression, resulting in progressive neurological deficits like hypoesthesia or motor weakness. Therapy is still variable. Aim of the study was to compare our surgical treatment and our follow-up regime including high-resolution nerve sonography with the current literature to evaluate best treatment of perineuriomas.MethodsRetrospective analysis of our dataset “peripheral nerve lesion” to identify patients suffering from perineuriomas between 01.01.2012 until 31.12.2018. Surgical treatment and the follow-up examination of three patients were described. Additionally, a systematic review including PubMed, the Cochrane Collaboration Library, Scopus and Google Scholar was performed for literature published between January 1, 1990 and October 31, 2019 independently by 2 authors.ResultsIn the first case, the left ulnar nerve was affected. In the second case, the left peroneal nerve and in the third case the right median nerve was affected. High-resolution nerve sonography was performed in each case. All patients underwent interfascicular neurolysis combined with a targeted fascicular biopsy under electrophysiological monitoring. Neurological deficits improved subsidized by rehabilitation. Surgical therapy and the neurological outcome were compared with literature. Systematic review revealed 22 articles, which met the inclusion criteria. Therefore, demographics, surgical treatment and neurological outcome of 77 patients were analysed.ConclusionsPerineuriomas are rare benign nerve sheath tumours with a slow progression, sometimes difficult to diagnose. Decompression and neurolysis may improve neurological deficits. High resolution nerve sonography might serve as a helpful additional diagnostic tool in this process.

Highlights

Perineuriomas are rare benign peripheral nerve sheath tumours of perineurial cell origin and can be classified into intraneural and extraneural perineuriomas
Case presentations Case 1 A 56-year-old male patient with typical clinical and electroneurographic findings of carpal-tunnelsyndrome caused by an intraneural PN of the right median nerve presented to our neurosurgical department
Electrophysiological testing revealed an impairment of the left ulnar nerve, high-resolution nerve sonography (HRNS) and magnetic resonance imaging (MRI) of the left arm showed intraneural PN of the ulnar nerve with a length of about 15 cm reaching from the middle of the upper arm to the middle of the forearm (Fig. 3)

Summary

Introduction

Perineuriomas are rare benign peripheral nerve sheath tumours of perineurial cell origin and can be classified into intraneural and extraneural perineuriomas. They most commonly present a mononeuropathy of gradual onset and slow progression, resulting in progressive neurological deficits like hypoesthesia or motor weakness. Perineuriomas (PN) are rare benign peripheral nerve sheath tumours originating from perineurial cells. The intraneural PN is restricted to the boundaries of the peripheral nerve, whereas the extraneural PN is found mainly in soft tissues and skin [1]. Alterations in NF1 or NF2 likely contribute to perineurioma pathogenesis, similar to other nerve sheath tumours [8]

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