Skip to main content

Advertisement

SpringerLink
Log in

Tumors of the peripheral nerves and plexuses

  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Opinion statement

Peripheral nerve tumors are a diverse group of lesions histologically and in their clinical behavior. The genetic disorders neurofibromatosis type 1 and 2 and schwannomatosis are significant risk factors for the development of peripheral nerve tumors. An understanding of these disorders is important in allowing appropriate management. Active treatment of peripheral nerve tumors is reserved for lesions that are malignant or causing neurologic dysfunction, pain, compressive symptomatology, or cosmetic concern. The mainstay of treatment is surgical intervention, the nature of which will vary with the type of tumor and anatomical location. In the case of malignant tumors, adjuvant chemotherapy and radiotherapy are commonly used. Developments in the understanding of the genetics and molecular biology of peripheral nerve tumors are opening up potentially exciting new avenues of treatment. The prognosis of benign peripheral nerve tumors is excellent, with a recurrence rate of just 5% or less after successful surgery. Malignant tumors have proven to be more challenging, with a much higher recurrence rate and a 5-year survival rate of 64%.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Harkin J, Reed RJ: Tumors of the Peripheral Nervous System. Washington DC: Armed Forces Institute of Pathology; 1969.

    Google Scholar 

  2. Kleihues P, Cavenee WK: World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: AZRC Press; 2000. Clearly describes the current pathological classification of PNTs, with a concise overview of each type.

    Google Scholar 

  3. Kransdorf M, Murphay MD: Neurogenic tumors. In Imaging of Soft Tissue Tumors. Philadelphia: Saunders; 1997:235–273.

    Google Scholar 

  4. Skovronsky DM, Oberholtzer JC: Pathologic classification of peripheral nerve tumors. Neurosurg Clin N Am 2004, 15:157–166.

    Article  PubMed  Google Scholar 

  5. Pilavaki M, Chourmouzi D, Kiziridou A, et al.: Imaging of peripheral nerve sheath tumors with pathologic correlation: pictorial review. Eur J Radiol 2004, 52:229–239.

    Article  PubMed  CAS  Google Scholar 

  6. Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol 1988, 45:575–578.

  7. Huang JH, Zaghloul K, Zager EL: Surgical management of brachial plexus region tumors. Surg Neurol 2004, 61:372–378.

    Article  PubMed  Google Scholar 

  8. Moukarbel RV, Sabri AN: Current management of head and neck schwannomas. Curr Opin Otolaryngol Head Neck Surg 2005, 13:117–122.

    Article  PubMed  Google Scholar 

  9. Ducatman BS, Scheithauer BW, Piepgras DG, et al.: Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer 1986, 57:2006–2021.

    Article  PubMed  CAS  Google Scholar 

  10. Collin C, Godbold J, Hajdu S, Brennan M: Localized extremity soft tissue sarcoma: an analysis of factors affecting survival. J Clin Oncol 1987, 5:601–612.

    PubMed  CAS  Google Scholar 

  11. Perrin RG, Guha A: Malignant peripheral nerve sheath tumors. Neurosurg Clin N Am 2004, 15:203–216.

    Article  PubMed  Google Scholar 

  12. Sorensen SA, Mulvihill JJ, Nielsen A: Long-term followup of von Recklinghausen neurofibromatosis. Survival and malignant neoplasms. N Engl J Med 1986, 314:1010–1015.

    Article  PubMed  CAS  Google Scholar 

  13. Kourea HP, Bilsky MH, Leung DH, et al.: Subdiaphragmatic and intrathoracic paraspinal malignant peripheral nerve sheath tumors: a clinicopathologic study of 25 patients and 26 tumors. Cancer 1998, 82:2191–2203.

    Article  PubMed  CAS  Google Scholar 

  14. Ducatman BS, Scheithauer BW: Postirradiation neurofibrosarcoma. Cancer 1983, 51:1028–1033.

    Article  PubMed  CAS  Google Scholar 

  15. Foley KM, Woodruff JM, Ellis FT, Posner JB: Radiationinduced malignant and atypical peripheral nerve sheath tumors. Ann Neurol 1980, 7:311–318.

    Article  PubMed  CAS  Google Scholar 

  16. Giannini C, Scheithauer BW, Jenkins RB, et al.: Soft-tissue perineurioma. Evidence for an abnormality of chromosome 22, criteria for diagnosis, and review of the literature. Am J Surg Pathol 1997, 21:164–173.

    Article  PubMed  CAS  Google Scholar 

  17. Woodruff JM: The pathology and treatment of peripheral nerve tumors and tumor-like conditions. CA Cancer J Clin 1993, 43:290–308.

    PubMed  CAS  Google Scholar 

  18. Wallace MR, Marchuk DA, Andersen LB, et al.: Type 1 neurofibromatosis gene: identification of a large transcript disrupted in three NF1 patients. Science 1990, 249:181–186.

    Article  PubMed  CAS  Google Scholar 

  19. Gutmann DH: Recent insights into neurofibromatosis type 1: clear genetic progress. Arch Neurol 1998, 55:778–780.

    Article  PubMed  CAS  Google Scholar 

  20. Seymour-Dempsey K, Andrassy RJ: Neurofibromatosis: implications for the general surgeon. J Am Coll Surg 2002, 195:553–563.

    Article  PubMed  Google Scholar 

  21. Korf BR: Clinical features and pathobiology of neurofibromatosis 1. J Child Neurol 2002, 17:573–577.

    PubMed  Google Scholar 

  22. Zoller M, Rembeck B, Akesson HO, Angervall L: Life expectancy, mortality and prognostic factors in neurofibromatosis type 1. A twelve-year follow-up of an epidemiological study in Goteborg, Sweden. Acta Derm Venereol 1995, 75:136–140.

    PubMed  CAS  Google Scholar 

  23. BrooksDG: The neurofibromatoses: hereditary predisposition to multiple peripheral nerve tumors. Neurosurg Clin N Am 2004, 15:145–155. Excellent review of the neurofibromatoses and their association with PNTs.

    Article  PubMed  Google Scholar 

  24. Evans DG, Sainio M, Baser ME: Neurofibromatosis type 2. J Med Genet 2000, 37:897–904.

    Article  PubMed  CAS  Google Scholar 

  25. Lim DJ, Rubenstein AE, Evans DG, et al.: Advances in neurofibromatosis 2 (NF2): a workshop report. J Neurogenet 2000, 14:63–106.

    PubMed  CAS  Google Scholar 

  26. Martuza RL, Eldridge R: Neurofibromatosis 2 (bilateral acoustic neurofibromatosis). N Engl J Med 1988, 318:684–688.

    Article  PubMed  CAS  Google Scholar 

  27. Huang JH, Simon SL, Nagpal S, et al.: Management of patients with schwannomatosis: report of six cases and review of the literature. Surg Neurol 2004, 62:353–361.

    Article  PubMed  Google Scholar 

  28. Jacoby LB, Jones D, Davis K, et al.: Molecular analysis of the NF2 tumor-suppressor gene in schwannomatosis. Am J Hum Genet 1997, 61:1293–1302.

    Article  PubMed  CAS  Google Scholar 

  29. MacCollin M, Woodfin W, Kronn D, Short MP: Schwannomatosis: a clinical and pathologic study. Neurology 1996, 46:1072–1079.

    PubMed  CAS  Google Scholar 

  30. Seppala MT, Sainio MA, Haltia MJ, et al.: Multiple schwannomas: schwannomatosis or neurofibromatosis type 2? J Neurosurg 1998, 89:36–41.

    Article  PubMed  CAS  Google Scholar 

  31. Kransdorf MJ, Jelinek JS, Moser RPJr, et al.: Soft-tissue masses: diagnosis using MR imaging. AJR Am J Roentgenol 1989, 153:541–547.

    PubMed  CAS  Google Scholar 

  32. Sundaram M, McGuire MH, Herbold DR: Magnetic resonance imaging of soft tissue masses: an evaluation of fifty-three histologically proven tumors. Magn Reson Imaging 1988, 6:237–248.

    Article  PubMed  CAS  Google Scholar 

  33. Maniker AH: Diagnostic steps, imaging, and electrophysiology. Neurosurg Clin N Am 2004, 15:133–144.

    Article  PubMed  Google Scholar 

  34. Kobayashi H, Kotoura Y, Hosono M, et al.: MRI and scintigraphic features of extraabdominal desmoid tumors. Clin Imaging 1997, 21:35–39.

    Article  PubMed  CAS  Google Scholar 

  35. Lucas JD, O’Doherty MJ, Wong JC, et al.: Evaluation of fluorodeoxyglucose positron emission tomography in the management of soft-tissue sarcomas. J Bone Joint Surg Br 1998, 80:441–447.

    Article  PubMed  CAS  Google Scholar 

  36. Ferner RE, Lucas JD, O’Doherty MJ, et al.: Evaluation of (18)fluorodeoxyglucose positron emission tomography ((18)FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry 2000, 68:353–357.

    Article  PubMed  CAS  Google Scholar 

  37. Howe FA, Filler AG, Bell BA, Griffiths JR: Magnetic resonance neurography. Magn Reson Med 1992, 28:328–338.

    Article  PubMed  CAS  Google Scholar 

  38. Gutmann DH, Aylsworth A, Carey JC, et al.: The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA 1997, 278:51–57.

    Article  PubMed  CAS  Google Scholar 

  39. FernerRE, Gutmann DH: International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res 2002, 62:1573–1577. A recent consensus statement regarding the management of MPNST, from a consortium of international experts in the field.

    PubMed  CAS  Google Scholar 

  40. Donner TR, Voorhies RM, Kline DG: Neural sheath tumors of major nerves. J Neurosurg 1994, 81:362–373.

    Article  PubMed  CAS  Google Scholar 

  41. Artico M: Benign neural sheath tumours of major nerves: characteristics in 119 surgical cases. Acta Neurochir (Wien) 1997, 139:1108–1116.

    Article  CAS  Google Scholar 

  42. Kline D, Hudson A: Nerve Injuries: Surgical Outcomes of Injuries, Entrapments and Tumors. Philadelphia: WB Saunders; 1995.

    Google Scholar 

  43. Baehring JM, Betensky RA, Batchelor TT: Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology 2003, 61:696–698.

    PubMed  Google Scholar 

  44. Huang JH, Weil RJ, Zager EL: Surgical management of common peripheral nerve tumors. Electronic publication update: Medlink; 2004.

  45. Angelov L, Davis A, O’Sullivan B, et al.: Neurogenic sarcomas: experience at the University of Toronto. Neurosurgery 1998, 43:56–64.

    Article  PubMed  CAS  Google Scholar 

  46. Tiel R, Kline D: Peripheral nerve tumors: surgical principles, approaches, and techniques. Neurosurg Clin N Am 2004, 15:167–175. Comprehensive review of the surgical approach to PNTs.

    Article  PubMed  Google Scholar 

  47. Santoro A, Tursz T, Mouridsen H, et al.: Doxorubicin versus CYVADIC versus doxorubicin plus ifosfamide in firstline treatment of advanced soft tissue sarcomas: a randomized study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. J Clin Oncol 1995, 13:1537–1545.

    PubMed  CAS  Google Scholar 

  48. Santoro A: Advanced soft tissue sarcoma: how many more trials with anthracyclines and ifosfamide? Ann Oncol 1999, 10:151–154.

    Article  PubMed  CAS  Google Scholar 

  49. Adjuvant chemotherapy for localised resectable softtissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-analysis Collaboration. Lancet 1997, 350:1647–1654. Meta analysis of 1568 patients indicating the benefits of chemotherapy treatment in managing soft tissue sarcoma.

  50. MD Consult. http://www.mdconsult.com. Accessed December 14, 2005.

  51. Khanfir K, Alzieu L, Terrier P, et al.: Does adjuvant radiation therapy increase loco-regional control after optimal resection of soft-tissue sarcoma of the extremities? Eur J Cancer 2003, 39:1872–1880.

    Article  PubMed  CAS  Google Scholar 

  52. Pollack A, Zagars GK, Goswitz MS, et al.: Preoperative vs. postoperative radiotherapy in the treatment of soft tissue sarcomas: a matter of presentation. Int J Radiat Oncol Biol Phys 1998, 42:563–572.

    Article  PubMed  CAS  Google Scholar 

  53. Robinson MH, Keus RB, Shasha D, Harrison LB: Is preoperative radiotherapy superior to postoperative radiotherapy in the treatment of soft tissue sarcoma? Eur J Cancer 1998, 34:1309–1316.

    Article  PubMed  CAS  Google Scholar 

  54. Leroy K, Dumas V, Martin-Garcia N, et al.: Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1: a clinicopathologic and molecular study of 17 patients. Arch Dermatol 2001, 137:908–913.

    PubMed  CAS  Google Scholar 

  55. Kourea HP, Cordon-Cardo C, Dudas M, et al.: Expression of p27(kip) and other cell cycle regulators in malignant peripheral nerve sheath tumors and neurofibromas: the emerging role of p27(kip) in malignant transformation of neurofibromas. Am J Pathol 1999, 155:1885–1891.

    PubMed  CAS  Google Scholar 

  56. Nielsen GP, Stemmer-Rachamimov AO, Ino Y, et al.: Malignant transformation of neurofibromas in neurofibromatosis 1 is associated with CDKN2A/p16 inactivation. Am J Pathol 1999, 155:1879–1884.

    PubMed  CAS  Google Scholar 

  57. Ferner RE, O’Doherty MJ: Neurofibroma and schwannoma. Curr Opin Neurol 2002, 15:679–684.

    Article  PubMed  Google Scholar 

  58. NIH Clinical Research Studies (protocol number: 05-C-0232). A phase 1 trial of peginterferon Alfa-2b (PEG-Intron) for plexiform neurofibromas. http:// clinicalstudies.info.nih.gov/detail/a_2005-c-0232.html. Accessed December 14, 2005.

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Hospital of the University of Pennsylvania, 3400 Spruce Street, Silverstein 3, 9104, Philadelphia, PA, USA

    Jason H. Huang MD

Authors
  1. Jason H. Huang MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Victoria E. Johnson MBChB
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Eric L. Zager MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Huang, J.H., Johnson, V.E. & Zager, E.L. Tumors of the peripheral nerves and plexuses. Curr Treat Options Neurol 8, 299–308 (2006). https://doi.org/10.1007/s11940-006-0020-z

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11940-006-0020-z

Keywords

Search

Navigation