Abstract
Introduction
Intraneural perineurioma is a rare peripheral nerve tumor of childhood and early adulthood. Patients demonstrate progressive muscle weakness and atrophy largely without sensory complaints.
Case
We report two children with perineurioma affecting the radial and femoral nerves. Electromyography (EMG), ultrasound, and 3-T MR imaging were important tools for localizing perineurioma and permitting its differentiation from other nerve lesions. The first patient underwent surgical excision of the perineurioma and a traditional nerve graft. At 10 months post-operative follow-up, she demonstrated no meaningful recovery of muscle strength compared to her pre-operative assessment. EMG did confirm axonal continuity indicating that reinnervation had occurred via the nerve graft. The second patient underwent a two-staged surgical procedure that included an end-to-side nerve transfer. At 18 months post-operative follow-up, she demonstrated mild improvement in muscle strength and EMG evidence of ongoing reinnervation.
Conclusion
The surgical management of perineurioma remains controversial, and reports of clinical recovery after nerve grafts and nerve transfers vary. Nerve transfers have been reported to provide superior results to traditional nerve grafting in adults with post-traumatic plexus injuries. The modest gain in strength of our patient who underwent a nerve transfer raises the question if this may also apply to patients with perineurioma. Additional studies will be required, which must also take into consideration that features of long-standing neuropathy (i.e., limb length discrepancy) have the potential to reduce the likelihood of reinnervation and clinical recovery.
Similar content being viewed by others
References
Macarenco RS, Ellinger F, Oliveira AM (2007) Perineurioma: a distinctive and underrecognized peripheral nerve sheath neoplasm. Arch Pathol Lab Med 131(4):625–636
Cortes W, Cheng J, Matloub HS (2005) Intraneural perineurioma of the radial nerve in a child. J Hand Surg [Am] 30(4):820–825
Mitsumoto H, Estes ML, Wilbourn AJ, Culver JE Jr (1992) Perineurial cell hypertrophic mononeuropathy manifesting as carpal tunnel syndrome. Muscle Nerve 15(12):1364–1368
Mauermann ML, Amrami KK, Kuntz NL, Spinner RJ, Dyck PJ, Bosch EP et al (2009) Longitudinal study of intraneural perineurioma—a benign, focal hypertrophic neuropathy of youth. Brain 132(Pt 8):2265–2276
Phillips LH, Persing JA, Vandenberg SR (1991) Electrophysiological findings in localized hypertrophic mononeuropathy. Muscle Nerve 14(4):335–341
Ostergaard JR, Smith T, Stausbol-Gron B (2009) Intraneural perineurioma of the sciatic nerve in early childhood. Pediatr Neurol 41(1):68–70
Hahn AF, Mauermann ML, Dyck PJ, Keegan BM (2007) A 16-year-old girl with progressive weakness of the left leg. Neurology 69(1):84–90
Boyanton BL Jr, Jones JK, Shenaq SM, Hicks MJ, Bhattacharjee MB (2007) Intraneural perineurioma: a systematic review with illustrative cases. Arch Pathol Lab Med 131(9):1382–1392
Lacour-Petit MC, Lozeron P, Ducreux D (2003) MRI of peripheral nerve lesions of the lower limbs. Neuroradiology 45(3):166–170
Nguyen D, Dyck PJ, Daube JR (2007) Intraneural perineurioma of the radial nerve visualized by 3.0 Tesla MRI. Muscle Nerve 36(5):715–720
Heilbrun ME, Tsuruda JS, Townsend JJ, Heilbrun MP (2001) Intraneural perineurioma of the common peroneal nerve. Case report and review of the literature. J Neurosurg 94(5):811–815
Gruen JP, Mitchell W, Kline DG (1998) Resection and graft repair for localized hypertrophic neuropathy. Neurosurgery 43(1):78–83
Simmons Z, Mahadeen ZI, Kothari MJ, Powers S, Wise S, Towfighi J (1999) Localized hypertrophic neuropathy: magnetic resonance imaging findings and long-term follow-up. Muscle Nerve 22(1):28–36
Lawson RD, Stewart DA, Tonkin MA (2015) Lesions of the hand. In: Farhadi RD, Bulstrode NW, Cugno S (eds). Plastic and reconstructive surgery: approaches and techniques, 1 edn. John WIley and Sons, Ltd, p 904–22
Iyer VG, Garretson HD, Byrd RP, Reiss SJ (1988) Localized hypertrophic mononeuropathy involving the tibial nerve. Neurosurgery 23(2):218–221
Alfonso DT, Sotrel A, Grossman JA (2001) Carpal tunnel syndrome due to an intraneural perineurioma in a 2-year-old child. J Hand Surg (Br) 26(2):168–170
Restrepo CE, Amrami KK, Howe BM, Dyck PJ, Mauermann ML, Spinner RJ (2015) The almost-invisible perineurioma. Neurosurg Focus 39(3), E13
Ahlawat S, Chhabra A, Blakely J (2014) Magnetic resonance neurography of peripheral nerve tumors and tumorlike conditions. Neuroimaging Clin N Am 24(1):171–192
Uetani M, Hashmi R, Hayashi K, Nagatani Y, Narabayashi Y, Imamura K (1998) Peripheral nerve intraneural ganglion cyst: MR findings in three cases. J Comput Assist Tomogr 22(4):629–632
Emory TS, Scheithauer BW, Hirose T, Wood M, Onofrio BM, Jenkins RB (1995) Intraneural perineurioma. A clonal neoplasm associated with abnormalities of chromosome 22. Am J Clin Pathol 103(6):696–704
Garg R, Merrel GA, Hillstrom HJ, Wolfe SW (2011) Comparison of nerve transfers and nerve grafting for traumatic upper plexus palsy: a systematic review and analysis. J Bone Joint Surg Am 93(9):819–829
Matthies C, Samii M (1997) Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation. Neurosurgery 40(1):1–9
Pitchford CW, Schwartz HS, Atkinson JB, Cates JM (2006) Soft tissue perineurioma in a patient with neurofibromatosis type 2: a tumor not previously associated with the NF2 syndrome. Am J Surg Pathol 30(12):1624–1629
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors confirm that they have no conflict of interest.
Rights and permissions
About this article
Cite this article
McMillan, H.J., Torres, C., Michaud, J. et al. Diagnosis and outcome of childhood perineurioma. Childs Nerv Syst 32, 1555–1560 (2016). https://doi.org/10.1007/s00381-016-3089-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-016-3089-z