Skip to main content

Advertisement

SpringerLink
Log in

Pediatric peripheral nerve tumors: clinical and surgical aspects

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

Pediatric peripheral nerve tumors (PNTs) are rare. Most are related to neurofibromatosis type 1 (NF1) with the potential for malignancy. An ongoing debate occurs about the best approach to such patients. This study describes a cohort of pediatric patients with PNTs and discusses clinical characteristics and surgical treatment.

Methods

We retrospectively reviewed the charts of seven pediatric patients with eight PNTs surgically treated from 2007 to 2018. Information concerning patient demographics, clinical presentation, PNTs characteristics, treatment choice, and outcome were recorded.

Results

All children presented with intense pain and a palpable mass. Three of the eight tumors were associated with a neurological deficit. Among the four patients with NF1, two had a neurofibroma and two a malignant peripheral nerve sheath tumor (MPNST). Histologically, three of the lesions were a benign peripheral nerve sheath tumor (BPNST), three a MPNST, and one each a desmoid tumor and Ewing’s sarcoma. Two of the eight tumors underwent partial tumor excision and six gross total excisions.

Conclusions

Intense pain at rest, day, and/or night, preventing normal activities; a palpable, hard, immobile mass; an intense Tinel’s sign related to the lump; clinical evidence of NF1; and high-speed growth of a tumor in the trajectory of the nerve or plexus should alert the clinician to the potential for malignancy. Preoperative biopsy is not indicated when clinical and imaging findings suggest a benign tumor. The surgical management of PNTs must be to achieve total resection, including wide margins with malignant tumors, though this is not always possible.

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

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Baccon J, Wellons JC, Rizk E (2015) Pediatric Peripheral Nerve Tumors. In: Tubbs RS, Rizk E, Shoja MM, Loukas M, Barbaro N, Spinner RJ (eds) Nerves and nerves injuries: pain, treatment, injury, disease and future directions, vol 2, 1st edn. Elsevier, pp 839–846

  2. Kim DH, Murovic JA, Tiel RL, Kline DG (2004) Operative outcomes of 546 Louisiana State University Health Sciences Center peripheral nerve tumors. Neurosurg Clin N Am 15:177–192. https://doi.org/10.1016/j.nec.2004.02.006

    Article  PubMed  Google Scholar 

  3. Coffin CM, Dehner LP (1989) Peripheral neurogenic tumors of the soft tissues in children and adolescents: a clinicopathologic study of 139 cases. Pediatr Pathol 9:387–407. https://doi.org/10.3109/15513818909022361

    Article  CAS  PubMed  Google Scholar 

  4. Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS (2014) Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. Pediatr Blood Cancer 61:1955–1960. https://doi.org/10.1002/pbc.25149

    Article  PubMed  Google Scholar 

  5. Topçu S, Alper A, Gülhan E, Koçyigit O, Tastepe I, Cetin G (2000) Neurogenic tumours of the mediastinum: a report of 60 cases. Can Respir J 7:261–265. https://doi.org/10.1155/2000/782325

    Article  PubMed  Google Scholar 

  6. Ferner RE, Gutmann DH (2002) International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res 62:1573–1577

    CAS  PubMed  Google Scholar 

  7. Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J (2005) Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol 23:8422–8430. Erratum in: J Clin Oncol 24:724. https://doi.org/10.1200/JCO.2005.01.4886

    Article  PubMed  Google Scholar 

  8. Sughrue ME, Levine J, Barbaro NM (2008) Pain as a symptom of peripheral nerve sheath tumors: clinical significance and future therapeutic directions. J Brachial Plex Peripher Nerve Inj 3:6–e140. https://doi.org/10.1186/1749-7221-3-6

    Article  PubMed  PubMed Central  Google Scholar 

  9. Aggarwal G, Satsangi B, Shukla S, Lahoti BK, Mathur RK, Maheshwari A (2010) Rare asymptomatic presentations of schwannomas in early adolescence: three cases with review of literature. Int J Surg 8:203–206. https://doi.org/10.1016/j.ijsu.2010.01.012

    Article  PubMed  Google Scholar 

  10. Guedes-Corrêa JF, Barbosa DAN (2015) Critical correlation between clinical presentation, imaging and type of peripheral nerve sheath tumors: a surgical approach. World Neurosurg 84:598. https://doi.org/10.1016/j.wneu.2015.05.041

    Article  Google Scholar 

  11. Dombi E, Ardern-Holmes SL, Babovic-Vuksanovic D, Barker FG, Connor S, Evans DG, Fisher MJ, Goutagny S, Harris GJ, Jaramillo D, Karajannis MA, Korf BR, Mautner V, Plotkin SR, Poussaint TY, Robertson K, Shih CS, Widemann BC, REiNS International Collaboration (2013) Recommendations for imaging tumor response in neurofibromatosis clinical trials. Neurology 81:S33–S40. https://doi.org/10.1212/01.wnl.0000435744.57038.af

    Article  PubMed  PubMed Central  Google Scholar 

  12. Friedrich RE, Hartmann M, Mautner VF (2007) Malignant peripheral nerve sheath tumors (MPNST) in NF1-affected children. Anticancer Res 27:1957–1960

    CAS  PubMed  Google Scholar 

  13. Binder DK, Smith JS, Barbaro NM (2004) Primary brachial plexus tumors: imaging, surgical, and pathological findings in 25 patients. Neurosurg Focus 16:E11. https://doi.org/10.3171/foc.2004.16.5.12

    Article  PubMed  Google Scholar 

  14. Nguyen R, Kluwe L, Fuensterer C, Kentsch M, Friedrich RE, Mautner VF (2011) Plexiform neurofibromas in children with neurofibromatosis type 1: frequency and associated clinical deficits. J Pediatr 159:652–655. https://doi.org/10.1016/j.jpeds.2011.04.008

    Article  PubMed  Google Scholar 

  15. Valeyrie-Allanore L, Ismaïli N, Bastuji-Garin S, Zeller J, Wechsler J, Revuz J, Wolkenstein P (2005) Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1. Br J Dermatol 153:79–82. https://doi.org/10.1111/j.1365-2133.2005.06558.x

    Article  CAS  PubMed  Google Scholar 

  16. Talwalkar SC, Cutler L, Stilwell JH (2005) Multiple plexiform schwannoma of the hand and forearm: a long-term follow-up. J Hand Surg Br 30:358–360. https://doi.org/10.1016/j.jhsb.2005.04.008

    Article  CAS  PubMed  Google Scholar 

  17. deCou JM, Rao BN, Parham DM, Lobe TE, Bowman L, Pappo AS, Fontanesi J (1995) Malignant peripheral nerve sheath tumors: the St. Jude Children’s Research Hospital experience. Ann Surg Oncol 2:524–529. https://doi.org/10.1007/BF02307086

    Article  CAS  PubMed  Google Scholar 

  18. Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A (2002) Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet 39:311–314. https://doi.org/10.1136/jmg.39.5.311

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Guedes F, Amorim R, Megali R, Hon Y (2011) Tumores de plexo braquial. In: Bijos PB, Guedes-Corrêa JF (eds) Plexo Braquial, 1st edn. DiLivros, Rio de Janeiro, pp 165–184

    Google Scholar 

  20. Secer HI, Daneyemez M, Gonul E, Izci Y (1976) Medical research council: aids to examination of the peripheral nervous system. Memorandum n° 45. Her Majesty's stationery office, London

    Google Scholar 

  21. Wewers ME, Lowe NK (1990) A critical review of visual analogue scales in the measurement of clinical phenomena. Res Nurs Health 13:227–236

    Article  CAS  Google Scholar 

  22. Cates JM, Coffin CM (2012) Neurogenic tumors of soft tissue. Pediatr Dev Pathol 15(1 Suppl):62–107. https://doi.org/10.2350/11-03-1003-PB.1

    Article  PubMed  Google Scholar 

  23. Thacker MM (2013) Benign soft tissue tumors in children. Orthop Clin North Am 44:433–444, xi. https://doi.org/10.1016/j.ocl.2013.05.001

    Article  PubMed  Google Scholar 

  24. Thacker MM (2013) Malignant soft tissue tumors in children. Orthop Clin North Am 44:657–667. https://doi.org/10.1016/j.ocl.2013.07.002

    Article  PubMed  Google Scholar 

  25. Amirian ES, Goodman JC, New P, Scheurer ME (2014) Pediatric and adult malignant peripheral nerve sheath tumors: an analysis of data from the surveillance, epidemiology, and end results program. J Neuro-Oncol 116:609–616. https://doi.org/10.1007/s11060-013-1345-6

    Article  Google Scholar 

  26. Ogose A, Hotta T, Morita T, Yamamura S, Hosaka N, Kobayashi H, Hirata Y (1999) Tumors of peripheral nerves: correlation of symptoms, clinical signs, imaging features, and histologic diagnosis. Skelet Radiol 28:183–188. https://doi.org/10.1007/s002560050498

    Article  CAS  Google Scholar 

  27. Pourtsidis A, Doganis D, Baka M, Bouhoutsou D, Varvoutsi M, Synodinou M, Giamarelou P, Kosmidis H (2014) Malignant peripheral nerve sheath tumors in children with neurofibromatosis type 1. Case Rep Oncol Med 2014:843749–843746. https://doi.org/10.1155/2014/843749

    Article  PubMed  PubMed Central  Google Scholar 

  28. Sørensen SA, Mulvihill JJ, Nielsen A (1986) Long-term follow-up of von Recklinghausen neurofibromatosis. Survival and malignant neoplasms. N Engl J Med 314:1010–1015. https://doi.org/10.1056/NEJM198604173141603

    Article  PubMed  Google Scholar 

  29. Rasmussen SA, Yang Q, Friedman JM (2001) Mortality in neurofibromatosis 1: an analysis using U.S. death certificates. Am J Hum Genet 68:1110–1118. https://doi.org/10.1086/320121

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Levi AD, Ross AL, Cuartas E, Qadir R, Temple HT (2010) The surgical management of symptomatic peripheral nerve sheath tumors. Neurosurgery 66:833–840. https://doi.org/10.1227/01.NEU.0000367636.91555.70

    Article  PubMed  Google Scholar 

  31. De Sousa ACS, Guedes-Correa JF, Costa JP, Amorim RMP, Santos LL, Pereira MRC (2014) Abstracts of the xviii international congress of neuropathology, Rio de Janeiro, Brazil, September 14 to 18, 2014 p23-03: preoperative biopsies in peripheral nerve tumors: clinical consequences. Brain Pathol 24(Suppl 1):1–103

    Google Scholar 

  32. Rodriguez FJ, Folpe AL, Giannini C, Perry A (2012) Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems. Acta Neuropathol 123:295–319. https://doi.org/10.1007/s00401-012-0954-z

    Article  PubMed  PubMed Central  Google Scholar 

  33. Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM (1986) Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer 57:2006–2021. https://doi.org/10.1002/1097-0142(19860515)57:10<2006::aid-cncr2820571022>3.0.co;2-6

    Article  CAS  PubMed  Google Scholar 

  34. Meis JM, Enzinger FM, Martz KL, Neal JA (1992) Malignant peripheral nerve sheath tumors (malignant schwannomas) in children. Am J Surg Pathol 16:694–707

    Article  CAS  Google Scholar 

  35. Takeda S, Miyoshi S, Minami M, Matsuda H (2004) Intrathoracic neurogenic tumors--50 years’ experience in a Japanese institution. Eur J Cardiothorac Surg 26:807–812. https://doi.org/10.1016/j.ejcts.2004.07.014

    Article  PubMed  Google Scholar 

  36. Korf BR (2000) Malignancy in neurofibromatosis type 1. Oncologist 5:477–485. https://doi.org/10.1634/theoncologist.5-6-477

    Article  CAS  PubMed  Google Scholar 

  37. Korf BR (1999) Plexiform neurofibromas. Am J Med Genet 89:31–37. https://doi.org/10.1002/(SICI)1096-8628(19990326)89:1<31::AID-AJMG7>3.0.CO;2-W

    Article  CAS  PubMed  Google Scholar 

  38. Grünewald TGP, Cidre-Aranaz F, Surdez D, Tomazou EM, de Álava E, Kovar H, Sorensen PH, Delattre O, Dirksen U (2018) Ewing sarcoma. Nat Rev Dis Primers 4:5. https://doi.org/10.1038/s41572-018-0003-x

    Article  PubMed  Google Scholar 

  39. Mitchell BD, Fox BD, Viswanathan A, Mitchell AH, Powell SZ, Cech DA (2010) Ewing sarcoma mimicking a peripheral nerve sheath tumor. J Clin Neurosci 17:1317–1319. https://doi.org/10.1016/j.jocn.2010.02.010

    Article  CAS  PubMed  Google Scholar 

  40. Mohan AT, Park DH, Jalgaonkar A, Alorjani M, Aston W, Briggs T (2011) Intra-neural Ewing’s sarcoma of the upper limb mimicking a peripheral nerve tumour. A report of 2 cases. J Plast Reconstr Aesthet Surg 64:e153–e156. https://doi.org/10.1016/j.bjps.2011.01.010

    Article  PubMed  Google Scholar 

  41. Lavorato A, Titolo P, Vincitorio F, Cofano F, Garbossa D (2019) Intraneural Ewing sarcoma of fibular nerve: case report, radiologic findings and review of literature. World Neurosurg 123:212–215. https://doi.org/10.1016/j.wneu.2018.12.043

    Article  PubMed  Google Scholar 

  42. Guha D, Davidson B, Nadi M, Alotaibi NM, Fehlings MG, Gentili F, Valiante TA, Tator CH, Tymianski M, Guha A, Zadeh G (2018) Management of peripheral nerve sheath tumors: 17 years of experience at Toronto Western Hospital. J Neurosurg 128:1226–1234. https://doi.org/10.3171/2017.1.JNS162292

    Article  PubMed  Google Scholar 

  43. Tiel R, Kline D (2004) Peripheral nerve tumors: surgical principles, approaches, and techniques. Neurosurg Clin N Am 15:167–175, vi. https://doi.org/10.1016/j.nec.2004.02.003

    Article  PubMed  Google Scholar 

  44. Prada CE, Rangwala FA, Martin LJ, Lovell AM, Saal HM, Schorry EK, Hopkin RJ (2012) Pediatric plexiform neurofibromas: impact on morbidity and mortality in neurofibromatosis type 1. J Pediatr 160:461–467. https://doi.org/10.1016/j.jpeds.2011.08.051

    Article  PubMed  Google Scholar 

  45. An HY, Hong KT, Kang HJ, Choi JY, Hong C, Kim HY, Choi TH, Kang CH, Kim HS, Cheon JE, Park SH, Park JD, Park KD, Shin HY (2017) Malignant peripheral nerve sheath tumor in children: a single-institute retrospective analysis. Pediatr Hematol Oncol 34:468–477. https://doi.org/10.1080/08880018.2017.1408730

    Article  CAS  PubMed  Google Scholar 

  46. Wanebo JE, Malik JM, VandenBerg SR, Wanebo HJ, Driesen N, Persing JÁ (1993) Malignant peripheral nerve sheath tumors. A clinicopathologic study of 28 cases. Cancer 71:1247–1253. https://doi.org/10.1002/1097-0142(19930215)71:4<1247::aid-cncr2820710413>3.0.co;2-s

    Article  CAS  PubMed  Google Scholar 

  47. Casanova M, Ferrari A, Spreafico F, Luksch R, Terenziani M, Cefalo G, Massimino M, Gandola L, Lombardi F, Fossati-Bellani F (1999) Malignant peripheral nerve sheath tumors in children: a single-institution twenty-year experience. J Pediatr Hematol Oncol 21:509–513. https://doi.org/10.1097/00043426-199911000-00012

    Article  CAS  PubMed  Google Scholar 

  48. Bergamaschi L, Bisogno G, Manzitti C, D’Angelo P, Milano GM, Scagnellato A, Cappelletti M, Chiaravalli S, Dall’Igna P, Alaggio R, Ruggiero A, Di Martino M, Affinita MC, Pierobon M, Garaventa A, Casanova M, Ferrari A (2018) Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors. Pediatr Blood Cancer 65. https://doi.org/10.1002/pbc.26816

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Surgery, Division of Neurosurgery, Gaffrée e Guinle University Hospital, School of Medicine, Federal University of Rio de Janeiro State (UNIRIO), 775 Mariz e Barros Street, Rio de Janeiro, RJ, Brazil

    Fernando Guedes, Rosana Siqueira Brown, Francisco José Lourenço Torrão-Junior & Rogério Martin Pires Amorim

  2. Department of Neurosurgery, Stanford University School of Medicine, Stanford, CA, USA

    Daniel A. N. Barbosa

  3. Department of Surgery, Division of Surgical Oncology, Gaffrée e Guinle University Hospital, School of Medicine, Federal University of Rio de Janeiro State (UNIRIO), 775 Mariz e Barros Street, Rio de Janeiro, RJ, Brazil

    Guilherme de Andrade Gagheggi Ravanini

Authors
  1. Fernando Guedes
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rosana Siqueira Brown
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Francisco José Lourenço Torrão-Junior
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Daniel A. N. Barbosa
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Guilherme de Andrade Gagheggi Ravanini
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Rogério Martin Pires Amorim
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Rosana Siqueira Brown.

Ethics declarations

Conflict of interest

The authors declare that they have no conflicts of interest.

Ethics approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (Comitê de Ética em Pesquisa do Hospital Universitário Gaffrée e Guinle 5258) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Guedes, F., Brown, R.S., Torrão-Junior, F.J.L. et al. Pediatric peripheral nerve tumors: clinical and surgical aspects. Childs Nerv Syst 35, 2289–2297 (2019). https://doi.org/10.1007/s00381-019-04306-w

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-019-04306-w

Keywords

Search

Navigation