SpringerLink
Log in

High-frequency ultrasound imaging findings in the diagnosis of segmental schwannomatosis of the ulnar nerve: case report and literature review

  • Case Report
  • Published:
Skeletal Radiology Aims and scope Submit manuscript

Abstract

Schwannomatosis is characterized by the development of multiple schwannomas without evidence of vestibular tumors. Segmental schwannomatosis is defined as being limited to one limb or five or fewer contiguous segments of the spine. We report a case of a 20-year-old male with the painful masses of the left upper extremity with associated numbness and paresthesia in the ulnar nerve distribution. The high-frequency ultrasound showed that the ulnar nerve fascicles were enlarged and expanded with beadlike growth. The patient underwent surgery twice and all the tumors were pathologically confirmed to be schwannomas. Together, the medical history, imaging, and pathology findings indicated the diagnosis of segmental schwannomatosis. By the imaging diagnostic tools, MRI is the most commonly used in assistance with diagnosis of segmental schwannomatosis while high-frequency ultrasonography is rare. In this paper, we discuss the value of high-frequency ultrasonography in the diagnosis of this rare disease. This case report provides a deeper understanding of segmental schwannomatosis and may help improve the accuracy of preoperative diagnosis.

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

Access this article

Log in via an institution

Price includes VAT (Thailand)

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

Data availability

All data, models, and code generated or used during the study appear in the submitted article.

Abbreviations

CDFI:

Color doppler flow imaging

CT:

Computed tomography

MPNST:

Malignant peripheral nerve sheath tumour

MRI:

Magnetic resonance imaging

NF-1:

Neurofibromatosis type 1

NF-2:

Neurofibromatosis type 2

US:

Ultrasonography

References

  1. Hébert-Blouin MN, Amrami KK, Scheithauer BW, Spinner RJ. Multinodular/plexiform (multifascicular) schwannomas of major peripheral nerves: An underrecognized part of the spectrum of schwannomas. J Neurosurg. 2010;112:372–82.

    Article  PubMed  Google Scholar 

  2. Schweitzer KM Jr, Adams SB Jr, Nunley JA 2nd. Multiple schwannomas of the posterior tibial nerve: a case series. Foot Ankle Int. 2013;34:607–11.

    Article  PubMed  Google Scholar 

  3. Forthman CL, Blazar PE. Nerve tumors of the hand and upper extremity. Hand Clin. 2004;20:233–42.

    Article  PubMed  Google Scholar 

  4. Gonzalvo A, Fowler A, Cook RJ, Little NS, Wheeler H, McDonald KL, Biggs MT. Schwannomatosis, sporadic schwannomatosis, and familial schwannomatosis: a surgical series with long-term follow-up. J Neurosurg. 2011;114:756–62.

    Article  PubMed  Google Scholar 

  5. Javalkar VK, Pigott T, Pal P, Findlay G. Multiple schwannomas: report of two cases. Eur Spine J. 2007;16:s287–92.

    Article  Google Scholar 

  6. Ogose A, Hotta T, Morita T, Otsuka H, Hirata Y. Multiple schwannomas in the peripheral nerves. J Bone Joint Surg Br. 1998;80:657–61.

    Article  CAS  PubMed  Google Scholar 

  7. Molina AR, Chatterton BD, Kalson NS, Fallowfifield ME, Khandwala AR. Multiple schwannomas of the upper limb related exclusively to the ulnar nerve in a patient with segmental schwannomatosis. J Plast Reconstr Aesthet Surg. 2013;66:e376–9.

    Article  PubMed  Google Scholar 

  8. Kim HJ, Han JK, So JW, Jo HD. Recurred Segmental Schwannomatosis Without Neurofibromatosis Type 2. Soonchunhyang Med Sci. 2016;22:163–6.

    Article  Google Scholar 

  9. Agaram NP, Prakash S, Antonescu CR. Deep-seated plexiform schwannoma: A pathologic study of 16 cases and comparative analysis with the superficial variety. Am J Surg Pathol. 2005;29:1042–8.

    Article  PubMed  Google Scholar 

  10. MacCollin M, Chiocca EA, Evans DG, et al. Diagnostic criteria for schwannomatosis. Neurology. 2005;64:1838–45.

    Article  CAS  PubMed  Google Scholar 

  11. Fornage BD. Sonography of peripheral nerves of the extremities. Radiol Med. 1993;85:162–7.

    CAS  PubMed  Google Scholar 

  12. Brown JM, Yablon CM, Morag Y, Brandon CJ, Jacobson JA. US of the Peripheral Nerves of the Upper Extremity: A Landmark Approach. Radiographics. 2016;36:452–63.

    Article  PubMed  Google Scholar 

  13. Klijanienko J, Caillaud JM, Lagacé R. Cytohistologic correlations in schwannomas (neurilemmomas), including “ancient”, cellular, and epithelioid variants. Diagn Cytopathol. 2006;34:517–22.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Diagnostic Ultrasound Center, The First Bethune Hospital of Jilin University, No.1, ** Zhang, Yunfeng Qi, Jie Du & Lirong Zhao

Authors
  1. Shuang Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. **gwen Han
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. ** Zhang
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Yunfeng Qi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Jie Du
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Lirong Zhao
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Lirong Zhao.

Ethics declarations

Informed consent

Informed consent was obtained from the subject described in this report.

Competing interests

The authors declare no competing interests.

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

Li, S., Han, J., Zhang, X. et al. High-frequency ultrasound imaging findings in the diagnosis of segmental schwannomatosis of the ulnar nerve: case report and literature review. Skeletal Radiol (2024). https://doi.org/10.1007/s00256-024-04645-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00256-024-04645-z

Keywords

Search

Navigation