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.

Keywords: Decompression and neurolysis; Intraneural perineurioma; Surgical treatment; Target fascicular biopsy.

Fig. 1

PRISMA flow chart demonstrating the systematic review of the literature

Fig. 2

HRNS (c + f) and MRI (a + d) of the right-hand depicted nerve enlargement (asterisk) with perineurial tissue and still identifiable fascicular structure. Intraoperative view showing the perineurioma (b + e)

Fig. 3

HRNS (b + c) and MRI (a + d) of the left forearm depicted nerve enlargement (asterisk) with perineurial tissue and still identifiable fascicular structure. Intraoperative view showing the perineurioma (e)

Fig. 4

HRNS (c + f) and MRI (a + d) of the left upper limb depicted nerve enlargement (asterisk) with perineurial tissue and still identifiable fascicular structure. Intraoperative view showing the perineurioma (b + e)

Fig. 5

Histology of the biopsy specimen (Hematoxylin and eosin stain) with intrafascicular tumour cells (asterisk) surrounded by collagen fibres (a). Epithelial membrane antigen staining is positive in tumour cells (asterisk) (b). S100 is not expressed in tumour cells but shows entrapped Schwann-cells in pseudo-onion bulbs (asterisk) (c). Immunohistochemical staining for Ki-67 reveals only very few proliferating tumour cells (asterisk) (d)