Perineurioma: A Rare Entity of Peripheral Nerve Sheath Tumors

Abstract

Objective Intraneural perineurioma is a rare tumor entity. It is a benign, very slow growing peripheral nerve sheath tumor that typically occurs in children and young adults. Motor deficits and muscle atrophy are classic presenting symptoms, while sensory deficits are rare at the onset of the disease. Recommended treatment strategies are lacking. We have evaluated the clinical follow-up and our experience with treatment of this rare entity.

Methods A total of 30 patients with intraneural perineuriomas were assessed retrospectively. Demographic data, clinical symptoms, diagnostic examinations, therapy strategies, and clinical outcome were analyzed. Descriptive statistical methods were used for evaluation.

Results The mean age was 22 years. Eleven women and 19 men were affected. The lesion occurred in the area of the upper extremity in 16 patients and in the area of the lower extremity in 14 patients. The most frequently affected nerve was the sciatic nerve, followed by the radial nerve. All patients showed a motor deficit to some extent. Seventy percent (n = 21) revealed atrophy, 43.3% (n = 13) had sensitive deficits, and 17% (n = 5) suffered of pain. Fascicle biopsies were performed in 26 patients (87%). In four patients (13%), the tumor was completely resected and then reconstructed via nerve grafts. Seventy percent of the patients (n = 21) received a magnetic resonance imaging (MRI) within 5 years postoperatively, in which no progress was shown.

Conclusions To diagnose perineurioma, it is essential to take a biopsy of an enlarged, nonfunctional fascicle. Furthermore, a long-distance epineuriotomy to decompress the hypertrophic fascicle is reasonable. To preserve the nerves' residual function, a complete resection is not recommended. Results after grafting are poor. One reason for this might be residual tumor cells along the nerve that cannot be visualized. Malignant transformation is not yet reported and tumor growth is stable for years.

Keywords

perineurioma - peripheral nerve sheath tumor - epineuriotomy - epithelial membrane antigen

Publikationsverlauf

Eingereicht: 13. April 2020

Angenommen: 28. Oktober 2020

Artikel online veröffentlicht:
24. Mai 2021

• References

• 1 Macarenco RS, Ellinger F, Oliveira AM. Perineurioma: a distinctive and underrecognized peripheral nerve sheath neoplasm. Arch Pathol Lab Med 2007; 131 (04) 625-636
• 2 Heilbrun ME, Tsuruda JS, Townsend JJ, Heilbrun MP. Intraneural perineurioma of the common peroneal nerve. Case report and review of the literature. J Neurosurg 2001; 94 (05) 811-815
• 3 Boyanton Jr BL, Jones JK, Shenaq SM, Hicks MJ, Bhattacharjee MB. Intraneural perineurioma: a systematic review with illustrative cases. Arch Pathol Lab Med 2007; 131 (09) 1382-1392
• 4 Mauermann ML, Amrami KK, Kuntz NL. et al. Longitudinal study of intraneural perineurioma: a benign, focal hypertrophic neuropathy of youth. Brain 2009; 132 (Pt 8): 2265-2276
• 5 McMillan HJ, Torres C, Michaud J, Ying Y, Boyd KU, Bourque PR. Diagnosis and outcome of childhood perineurioma. Childs Nerv Syst 2016; 32 (08) 1555-1560
• 6 Restrepo CE, Amrami KK, Howe BM, Dyck PJB, Mauermann ML, Spinner RJ. The almost-invisible perineurioma. Neurosurg Focus 2015; 39 (03) E13
• 7 León Cejas L, Binaghi D, Socolovsky M. et al. Intraneural perineuriomas: diagnostic value of magnetic resonance neurography. J Peripher Nerv Syst 2018; 23 (01) 23-28
• 8 Scheller C, Richter HP, Scheuerle A, Kretschmer T, König RW, Antoniadis G. Intraneural perineuriomas; a rare entity. Clinical, surgical and neuropathological details in the management of these lesions. Zentralbl Neurochir 2008; 69 (03) 134-138
• 9 Wilson TJ, Howe BM, Stewart SA, Spinner RJ, Amrami KK. Clinicoradiological features of intraneural perineuriomas obviate the need for tissue diagnosis. J Neurosurg 2018; 129 (04) 1034-1040
• 10 Gruen JP, Mitchell W, Kline DG. Resection and graft repair for localized hypertrophic neuropathy. Neurosurgery 1998; 43 (01) 78-83