SpringerLink
Log in

Brachial plexus peripheral nerve sheath tumors (PNSTs): clinical and surgical management in the pediatric population

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

Abstract

Purpose

Peripheral nerve sheath tumors (PNSTs) are rare in pediatric patients, especially in the brachial plexus. Research on PNSTs is lacking. This article presents a retrospective cohort study of pediatric patients diagnosed and treated with PNSTs, specifically brachial plexus tumors.

Methods

All pediatric patients intervened in a single center between 2007 and 2023 with brachial plexus tumors were systemically analyzed.

Results

Eleven pediatric patients with 14 brachial plexus PNSTs were studied. The gender distribution was 64% female and 36% male, with an average age of 10.7 years. Ninety-one percent had a previous NF-1 diagnosis. Right brachial plexus presented a higher prevalence (64%). Pain, Tinel’s sign, and stiffness masses were common during diagnosis. Motor deficits were noted in 43% of the patients. Surgery was indicated for symptoms, particularly pain and rapid growth, increasing malignancy risk. Due to suspected malignancy, an en bloc resection with safety margins was performed. Among the patients, 57% received a histopathological diagnosis of MPNST (malignant peripheral nerve sheath tumor). Treatment included radiotherapy and chemotherapy. Clinical follow-up was conducted for all cases, involving clinical and oncological evaluations for all MPNSTs.

Conclusions

This article present a series of pediatric brachial plexus tumors, especially in NF-1, and emphasizes the importance of thorough evaluation for this group. Swift diagnosis is crucial in pediatrics, enabling successful surgery for small lesions with limited neurological symptoms, improving long-term outcomes. Prompt referral to specialized services is urged for suspected masses, irrespective of neurological symptoms. Benign tumor postsurgical progression shows better outcomes than MPNSTs, with complete resection as the primary goal. Needle-guided biopsy is not recommended.

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

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price includes VAT (France)

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

Data availability

No datasets were generated or analysed during the current study.

Abbreviations

PNST:

Peripheral nerve sheath tumors

BP:

Brachial plexus

BPTs:

Brachial plexus tumors

PAS:

Pain assessment scale

BPNSTs:

Benignant tumors of the nerve sheath

MPNSTs:

Malignant tumors of the nerve sheath

PP:

Pediatric population

NF-1:

Neurofibromatosis type 1

NF-2:

Neurofibromatosis type 2

NRSTS:

Nonrhabdomyosarcoma soft tissue sarcoma

GH:

Growth hormone

MAPK:

Mitogen-activated protein kinase

MRI:

Magnetic resonance imaging

STIR:

Short T1 inversion recovery

STS:

Soft tissue sarcomas

SUV:

Standardized uptake value

References

  1. Guedes F, Socolovsky M, Rasulic L, Garozzo D, Zager EL (2022) Diagnostic assessment and treatment of peripheral nerve tumors. Springer, Cham

    Google Scholar 

  2. DeCou JM, Rao BN, Parham DM et al (1995) Malignant peripheral nerve sheath tumors: the St. Jude children’s research hospital experience. Ann Surg Oncol 2(6):524–529. https://doi.org/10.1007/BF02307086

    Article  CAS  PubMed  Google Scholar 

  3. Yaworski A, Koujok K, Cheung K, Ying Y, McMillan HJ (2021) Intrinsic peripheral nerve and root tumor and pseudotumoral lesions at a tertiary care pediatric hospital. Childs Nerv Syst 37(4):1229–1236. https://doi.org/10.1007/s00381-020-04995-8

    Article  PubMed  Google Scholar 

  4. Costales JR, Socolovsky M, Sánchez Lázaro JA, ÁlvarezGarcía R, Costales DR (2019) Peripheral nerve injuries in the pediatric population: a review of the literature. Part III: peripheral nerve tumors in children. Childs Nerv Syst 35(1):47–52. https://doi.org/10.1007/s00381-018-3976-6

    Article  PubMed  Google Scholar 

  5. Zipfel J, Al-Hariri M, Gugel I et al (2020) Surgical management of peripheral nerve sheath tumors in children, with special consideration of neurofibromatoses [published correction appears in Childs Nerv Syst. 2021 Sep;37(9):2953]. Childs Nerv Syst 36(10):2433-2442https://doi.org/10.1007/s00381-020-04703-6

  6. 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(11):1955–1960. https://doi.org/10.1002/pbc.25149

    Article  PubMed  Google Scholar 

  7. Siqueira MG, Martins RS, Teixeira MJ (2009) Management of brachial plexus region tumours and tumour-like conditions: relevant diagnostic and surgical features in a consecutive series of eighteen patients. Acta Neurochir (Wien) 151(9):1089–1098. https://doi.org/10.1007/s00701-009-0380-8

    Article  CAS  PubMed  Google Scholar 

  8. 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(2):177–192. https://doi.org/10.1016/j.nec.2004.02.006

    Article  PubMed  Google Scholar 

  9. Gosk J, Rutowski R, Zimmer K, Rabczyński J (2004) Brachial plexus tumours–own experience in diagnostics and surgical treatment. Folia Neuropathol 42(3):171–175

    PubMed  Google Scholar 

  10. 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(4):387–407. https://doi.org/10.3109/15513818909022361

    Article  CAS  PubMed  Google Scholar 

  11. Donner TR, Voorhies RM, Kline DG (1994) Neural sheath tumors of major nerves. J Neurosurg 81(3):362–373. https://doi.org/10.3171/jns.1994.81.3.0362

    Article  CAS  PubMed  Google Scholar 

  12. Mackinnon SE (2015) Nerve Surgery. Thieme, New York (Chapter 18)

    Book  Google Scholar 

  13. Dunning-Davies BM, Parker AP (2016) Annual review of children with neurofibromatosis type 1. Arch Dis Child Educ Pract Ed 101(2):102–111. https://doi.org/10.1136/archdischild-2014-308084

    Article  CAS  PubMed  Google Scholar 

  14. Korf BR (1999) Plexiform neurofibromas. Am J Med Genet 89(1):31–37. https://doi.org/10.1002/(sici)1096-8628(19990326)89:1%3c31::aid-ajmg7%3e3.0.co;2-w

    Article  CAS  PubMed  Google Scholar 

  15. Gabhane SK, Kotwal MN, Bobhate SK (2009) Morphological spectrum of peripheral nerve sheath tumors: a series of 126 cases. Indian J Pathol Microbiol 52(1):29–33. https://doi.org/10.4103/0377-4929.44958

    Article  PubMed  Google Scholar 

  16. Rodriguez FJ, Stratakis CA, Evans DG (2012) Genetic predisposition to peripheral nerve neoplasia: diagnostic criteria and pathogenesis of neurofibromatoses, Carney complex, and related syndromes. Acta Neuropathol 123(3):349–367. https://doi.org/10.1007/s00401-011-0935-7

    Article  PubMed  Google Scholar 

  17. Farid M, Demicco EG, Garcia R et al (2014) Malignant peripheral nerve sheath tumors. Oncologist 19(2):193–201. https://doi.org/10.1634/theoncologist.2013-0328

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Reilly KM, Kim A, Blakely J et al (2017) Neurofibromatosis type 1-associated MPNST state of the science: outlining a research agenda for the future. J Natl Cancer Inst 109(8):djx124. https://doi.org/10.1093/jnci/djx124

    Article  PubMed  PubMed Central  Google Scholar 

  19. Carli M, Ferrari A, Mattke A et al (2005) Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group [published correction appears in J Clin Oncol. 2006 Feb 1;24(4):724. Koscielniak, Eura [corrected to Koscielniak, Ewa]]. J Clin Oncol 23(33):8422–8430. https://doi.org/10.1200/JCO.2005.01.4886

  20. Guedes F, Brown RS, Torrão-Junior FJL, Barbosa DAN, Ravanini GAG, Amorim RMP (2019) Pediatric peripheral nerve tumors: clinical and surgical aspects. Childs Nerv Syst 35(12):2289–2297. https://doi.org/10.1007/s00381-019-04306w

    Article  PubMed  Google Scholar 

  21. Cunha KS, Barboza EP, Fonseca EC (2008) Identification of growth hormone receptor in plexiform neurofibromas of patients with neurofibromatosis type 1. Clinics (Sao Paulo) 63(1):39–42. https://doi.org/10.1590/s1807-59322008000100008

    Article  PubMed  Google Scholar 

  22. Mazal AT, Faramarzalian A, Samet JD, Gill K, Cheng J, Chhabra A (2020) MR neurography of the brachial plexus in adult and pediatric age groups: evolution, recent advances, and future directions. Expert Rev Med Devices 17(2):111–122. https://doi.org/10.1080/17434440.2020.1719830

    Article  CAS  PubMed  Google Scholar 

  23. Stull MA, Moser RP Jr, Kransdorf MJ, Bogumill GP, Nelson MC (1991) Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol 20(1):9–14. https://doi.org/10.1007/BF00243714

    Article  CAS  PubMed  Google Scholar 

  24. Wasa J, Nishida Y, Tsukushi S et al (2010) MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol 194(6):1568–1574. https://doi.org/10.2214/AJR.09.2724

    Article  PubMed  Google Scholar 

  25. Brenner W, Friedrich RE, Gawad KA et al (2006) Prognostic relevance of FDG PET in patients with neurofibromatosis type-1 and malignant peripheral nerve sheath tumours. Eur J Nucl Med Mol Imaging 33(4):428–432. https://doi.org/10.1007/s00259-005-0030-1

    Article  PubMed  Google Scholar 

  26. Azizi AA, Slavc I, Theisen BE et al (2018) Monitoring of plexiform neurofibroma in children and adolescents with neurofibromatosis type 1 by [18 F]FDG-PET imaging. Is it of value in asymptomatic patients? Pediatr Blood Cancer 65(1). https://doi.org/10.1002/pbc.26733. https://doi.org/10.1002/pbc.26733

  27. Derlin T, Tornquist K, Münster S et al (2013) Comparative effectiveness of 18F-FDG PET/CT versus whole-body MRI for detection of malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Clin Nucl Med 38(1):e19–e25. https://doi.org/10.1097/RLU.0b013e318266ce84

    Article  PubMed  Google Scholar 

  28. Broski SM, Johnson GB, Howe BM et al (2016) Evaluation of (18)F-FDG PET and MRI in differentiating benign and malignant peripheral nerve sheath tumors. Skeletal Radiol 45(8):1097–1105. https://doi.org/10.1007/s00256-016-2394-7

    Article  PubMed  Google Scholar 

  29. Dombi E, Baldwin A, Marcus LJ et al (2016) Activity of selumetinib in neurofibromatosis type 1-related plexiform neurofibromas. N Engl J Med 375(26):2550–2560. https://doi.org/10.1056/NEJMoa1605943

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Gross AM, Wolters PL, Dombi E, et al (2020) Selumetinib in children with inoperable plexiform neurofibromas [published correction appears in N Engl J Med. 2020 Sep 24;383(13):1290]. N Engl J Med 382(15):1430–1442. https://doi.org/10.1056/NEJMoa1912735

  31. Das S, Ganju A, Tiel RL, Kline DG (2007) Tumors of the brachial plexus. Neurosurg Focus 22(6):E26. Published 2007 Jun 15. https://doi.org/10.3171/foc.2007.22.6.27

  32. Martin E, Flucke UE, Coert JH, van Noesel MM (2020) Treatment of malignant peripheral nerve sheath tumors in pediatric NF1 disease. Childs Nerv Syst 36(10):2453–2462. https://doi.org/10.1007/s00381-020-04687-3

    Article  PubMed  PubMed Central  Google Scholar 

  33. Lu VM, Wang S, Daniels DJ, Spinner RJ, Levi AD, Niazi TN (2021) The clinical course and role of surgery in pediatric malignant peripheral nerve sheath tumors: a database study. J Neurosurg Pediatr 29(1):92–99. Published 2021 Oct 8. https://doi.org/10.3171/2021.7.PEDS21263

  34. Olsen SH, Thomas DG, Lucas DR (2006) Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma. Mod Pathol 19(5):659–668. https://doi.org/10.1038/modpathol.3800569

    Article  CAS  PubMed  Google Scholar 

  35. Klijanienko J, Caillaud JM, Lagacé R, Vielh P (2002) Cytohistologic correlations of 24 malignant peripheral nerve sheath tumor (MPNST) in 17 patients: the Institut Curie experience. Diagn Cytopathol 27(2):103–108. https://doi.org/10.1002/dc.10152

    Article  PubMed  Google Scholar 

  36. Priya D, Sadasivan B, PatilOkaly GV, MukundaPai M, Alashetty S, Kavitha BL (2021) Cytomorphological and immunohistochemical features of renal and extrarenal rhabdoid tumors. Diagn Cytopathol 49(6):711–717. https://doi.org/10.1002/dc.24727

    Article  Google Scholar 

  37. Milbury CA, Creeden J, Yip WK et al (2022) Clinical and analytical validation of FoundationOne®CDx, a comprehensive genomic profiling assay for solid tumors. PLoS One 17(3):e0264138. Published 2022 Mar 16. https://doi.org/10.1371/journal.pone.0264138

  38. Pinet S, Durand S, Perani A, et al (2023) Clinical management of molecular alterations identified by high throughput sequencing in patients with advanced solid tumors in treatment failure: real-world data from a French hospital. Front Oncol 13:1104659. Published 2023 Feb 27. https://doi.org/10.3389/fonc.2023.1104659

  39. Guedes F, Henriques VM, Torrão FL et al (2024) When biopsy goes wrong: a case series of misdiagnoses and complications from biopsies of masses of unknown origin potentially originating from a peripheral nerve. J Neurosurg 140:480–488. Published online August 18, 2023. https://doi.org/10.3171/2023.5.JNS23385

  40. Kahn J, Gillespie A, Tsokos M et al (2014) Radiation therapy in management of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Front Oncol 4:324. Published 2014 Nov 17. https://doi.org/10.3389/fonc.2014.00324

  41. van Noesel MM, Orbach D, Brennan B et al (2019) Outcome and prognostic factors in pediatric malignant peripheral nerve sheath tumors: an analysis of the European Pediatric Soft Tissue Sarcoma Group (EpSSG) NRSTS-2005 prospective study. Pediatr Blood Cancer 66(10):e27833. https://doi.org/10.1002/pbc.27833

    Article  CAS  PubMed  Google Scholar 

  42. Ferrari A, Bisogno G, Carli M (2007) Management of childhood malignant peripheral nerve sheath tumor. Paediatr Drugs 9(4):239–248. https://doi.org/10.2165/00148581-200709040-00005

    Article  PubMed  Google Scholar 

  43. Lv S, Wang Q, Zhao W et al (2017) A review of the postoperative lymphatic leakage. Oncotarget 8(40):69062–69075. Published 2017 Apr 20. https://doi.org/10.18632/oncotarget.17297

  44. Bergqvist C, Servy A, Valeyrie-Allanore L et al (2020) Neurofibromatosis 1 French national guidelines based on an extensive literature review since 1966. Orphanet J Rare Dis 15(1):37. Published 2020 Feb 3. https://doi.org/10.1186/s13023-020-1310-3

Download references

Author information

Authors and Affiliations

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

    Fernando Guedes, Evelina Llorian, Vinícius M. Henriques & Francisco José Lourenço Torrão-Junior

Authors
  1. Fernando Guedes
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Evelina Llorian
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Vinícius M. Henriques
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Francisco José Lourenço Torrão-Junior
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

F.G made the draft. E.L.,V.H. and F.G wrote the main manuscript text. E.L, V.H and F.T data collection and data analysis. F.G, E.L, V.H bibliographic collection. F.G and F.T. edition. All authors reviewed the manuscript.

Corresponding author

Correspondence to Fernando Guedes.

Ethics declarations

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 and Guinle 5258) and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Conflict of interest

The authors declare that they have no conflicts of interest.

Additional information

Publisher's Note

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

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Guedes, F., Llorian, E., Henriques, V.M. et al. Brachial plexus peripheral nerve sheath tumors (PNSTs): clinical and surgical management in the pediatric population. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06509-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00381-024-06509-2

Keywords

Search

Navigation