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

Abstract

Background: 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. 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. Methods: Retrospective 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. Results: In 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. Conclusions: Perineuriomas 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.

A Rare case of Ulnar Nerve Intraneural Perineurioma in an elderly gentleman

“… 9 MRI shows a typically fusiform swelling of nerve fascicles with isointensity on T1 and hyperintensity on T2 with frequently a ‘target sign’ showing hyperintense periphery compared to the centre of lesion. 2 …”

“…recommended that extensive nerve resection with grafting should only be considered in distal lesions without muscle atrophy, as it may worsen disability and pain in proximal lesions. 2 As an alternative, Sachanandani et al. recommended distal nerve transfer to decrease regeneration length and eliminate secondary operative site.…”

“…However, according to the systematic review of all surgical treatment options, the neurological outcome did not differ. 2 The authors have suggested that there may a shift from total resection towards a wait-and-see approach following the targeted fascicular biopsy.…”

Acral perineurioma: a case report of a rare neoplasm successfully treated with autologous skin graft reconstructive surgery

“…PN is a rare nerve sheath tumor that originates exclusively from perineural cells, which take part in the blood-nerve barrier surrounding peripheral nerves. 1 Depending on the relationship with the nerve enveloped, PNs are classified into intraneural and extraneural ones, with different clinical presentation and etiology. 2 …”

Rare Tumors Causing Median Nerve Compression in Adults—A Narrative Review

“…The presentation is in most cases as a slow progressive mononeuropathy causing hypoesthesia and motor weakness. 9 No consensus about therapy exists, varying from…”