Skip to main content

Advertisement

SpringerLink
Log in

On Pseudo-Onion Bulb Intraneural Proliferations of the Non-Major Nerves of the Oral Mucosa

  • Original Paper
  • Published:
Head and Neck Pathology Aims and scope Submit manuscript

Abstract

Perineurial cells (PCs) participate in reactive and neoplastic processes, of the latter pure perineurial being intraneural (IP) and soft tissue perineuriomas with oral examples being reported in both. In our review of over 500 peripheral nerve sheath tumors including granular cell tumor, we identified a single ostensible case of IP occurring on the tongue of a 45-year-old African-American male that was characterized by classic perineurial pseudo-onion bulbs (PsOb), proliferating PCs among these PsOb, sclerosis apparently due to long term duration and a plexiform pattern. We have also encountered 37 examples of apparently reactive, hyperplastic or traumatic, PsOb intraneural pseudoperineuriomatous proliferation (IPP) simulating microscopically some of the properties of IP. The majority of the lesions occurred in women and close to 80 % affected the tongue. Three microscopic patterns were appreciated. Type I lesions were those where IPP was seen only focally, type II where it was seen in roughly half of the lesion, and type III where the majority of the lesional tissue or the lesion itself was characterized by IPP. Immunohistochemically, IPP featured PsOb with generally a single layer of PCs decorated by epithelial membrane antigen, glut-1 or claudin-1, and decreased numbers of S-100 positive Schwann cells. The number of axons was not apparently altered. A prominent collagenous intraneural component was occasionally evident among PsOb and the affected nerve featured discontinuous or absent perineurial envelop. While type I and II IPP can be distinguished from IP, the distinction from type III lesions can be problematic. However, the discontinuity of the perineurium of the affected nerve, the spacing and collagenization among PsOb, the limited perineurial cell layer defining the pseudo-onion bulbs, the absence of proliferating PCs between PsObs and the decreasing number of Schwann cells may be of help in the distinction from IP.

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
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10

Similar content being viewed by others

References

  1. Key A, Retzius G. Studien in der Anatomie des Nervensystemes. Arch Mikr Anat. 1873;9:308–86.

    Article  Google Scholar 

  2. Parmantier E, Lynn B, Lawson D, et al. Schwann cell-derived desert hedgehog controls the development of peripheral nerve sheaths. Neuron. 1999;23(4):713–24.

    Article  PubMed  CAS  Google Scholar 

  3. Hornick JL, Bundock EA, Fletcher CDM. Hybrid schwannoma/perineurioma: clinicopathologic analysis of 42 distinctive benign nerve sheath tumors. Am J Surg Pathol. 2009;33:1554–61.

    Article  PubMed  Google Scholar 

  4. Scheithauer BW, Woodruff JM, Erlandson RA. Miscellaneous benign neurogenic tumors. In: Rosai J, Sobin LH, editors. Atlas of tumor pathology—Tumors of peripheral nervous system. Washington DC: Third Series, Fascicle 24. Armed Forces Institute of Pathology; 1999. p. 219–82.

    Google Scholar 

  5. Boyanton BL Jr, Jones JK, Shenaq SM, Hicks MJ, Bhattacharjee MB. Intraneural perineurioma: a systematic review with illustrative cases. Arch Pathol Lab Med. 2007;131(9):1382–92.

    PubMed  Google Scholar 

  6. Mauerman ML, Amrami KK, Kuntz NL, et al. Longitudinal study of intraneural perineurioma—a benign, focal hypertrophic neuropathy of youth. Brain. 2009;132:2265–76.

    Article  Google Scholar 

  7. Huguet P, de la Torre J, Pallares J, et al. Intraosseous intraneural perineurioma: report of a case with morphological, immunohistochemical and FISH study. Med Oral. 2004;9(1):64–8.

    PubMed  Google Scholar 

  8. McNamara K, Mallery S, Kalmar J, Evans E. Central intraneural perineurioma of the mandible: a case report and review of the literature (Abstract #229). Annual Meeting, American Academy of Oral and Maxillofacial Pathology, San Francisco, CA 2008.

  9. Damm DD, White DK, Merrell JD. Intraneural perineurioma—not restricted to major nerves. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003;96(2):192–6.

    Article  PubMed  Google Scholar 

  10. da Cruz Perez DE, Amanajas de Aguiar FC Jr, Leon JE, Graner E, Paes de Almeida O, Vargas PA. Intraneural perineurioma of the tongue: a case report. J Oral Maxillofac Surg. 2006;64(7):1140–2.

    Article  PubMed  Google Scholar 

  11. Rocha LA, Lopes SM, Silva AR, Lopes MA, Vargas PA. Oral intraneural perineurioma. Report of two cases. Clinics (Sao Paolo). 2009;64:1037–9.

    Article  Google Scholar 

  12. Dundr P, Povysil C, Tvrdik D, Mazanek J. Intraneural perineurioma of the oral mucosa. Br J Oral Maxillofac Surg. 2007;45(6):503–4.

    Article  PubMed  Google Scholar 

  13. Siponen M, Sandor GK, Ylikontiola L, Salo T, Tuominen H. Multiple orofacial intraneural perineuriomas in a patient with hemifacial hyperplasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;104(1):e38–44.

    Article  PubMed  Google Scholar 

  14. Vashisht K, Rock RW, Summers BA. Multiple masses in a horse’s tongue resulting from an atypical perineurial cells proliferative disorder. Vet Pathol. 2007;44:398–402.

    Article  PubMed  CAS  Google Scholar 

  15. de La Jarte-Thirouard AS, Jacquier I, de Saint-Maur PP. Intraneural reticular perineurioma of the neck. Ann Diagn Pathol. 2003;7(2):120–3.

    Google Scholar 

  16. Santos-Briz A, Godoy E, Cañueto J, García JL, Mentzel T. Cutaneous intraneural perineurioma: a case report. Am J Dermapathol. 2013;35:e45-8.

    Google Scholar 

  17. da Gama Imaginário J, Coelho B, Tome F, Luis ML. Nevrite interstitielle hypertrophique monosymptomatique. J Neurol Sci. 1964;64:340–7.

    Article  Google Scholar 

  18. Mitsumoto H, Wilbourn AJ, Goren H. Perineurioma as the cause of localized hypertrophic neuropathy. Muscle Nerve. 1980;3(5):403–12.

    Article  PubMed  CAS  Google Scholar 

  19. Pina-Oviedo S, Ortiz-Hidalgo C. The normal and neoplastic perineurium: a review. Adv Anat Pathol. 2008;15(3):147–64.

    Article  PubMed  Google Scholar 

  20. Johnson PC, Kline DG. Localized hypertrophic neuropathy: possible focal perineurial barrier defect. Acta Neuropathol. 1989;77(5):514–8.

    Article  PubMed  CAS  Google Scholar 

  21. Emory TS, Scheithauer BW, Hirose T, Wood M, Onofrio BM, Jenkins RB. Intraneural perineurioma. A clonal neoplasm associated with abnormalities of chromosome 22. Am J Clin Pathol. 1995;103(6):696–704.

    PubMed  CAS  Google Scholar 

  22. Korthals JK, Gieron MA, Wisniewski HM. Nerve regeneration patterns after acute ischemic injury. Neurology. 1989;39:932–7.

    Article  PubMed  CAS  Google Scholar 

  23. Mirsky R, Parmantier E, McMahon AP, Jessen KR. Schwann cell-derived desert hedgehog signals nerve sheath formation. Ann N Y Acad Sci. 1999;883:196–202.

    Article  PubMed  CAS  Google Scholar 

  24. Chou SM. Immunohistochemical and ultrastructural classification of peripheral neuropathies with onion-bulbs. Clin Neuropathol. 1992;11(3):109–14.

    PubMed  CAS  Google Scholar 

  25. Lasota J, Fetsch JF, Wozniak A, Wasag B, Sciot R, Miettinen M. The neurofibromatosis type 2 gene is mutated in perineurial cell tumors: a molecular genetic study of eight cases. Am J Pathol. 2001;158:1223–9.

    Article  PubMed  CAS  Google Scholar 

  26. Koutlas IG, Scheithauer BW, Folpe AL. Intraoral perineurioma, soft tissue type: report of five cases, including 3 intraosseous examples, and review of the literature. Head Neck Pathol. 2010;4(2):113–20.

    Article  PubMed  Google Scholar 

  27. Weidner N, Nasr A, Johnston J. Plexiform soft tissue tumor composed predominantly of perineurial fibroblasts (perineurioma). Ultrastruct Pathol. 1993;17:251–62.

    Article  PubMed  CAS  Google Scholar 

  28. Zelger B, Weinlich G, Perineuroma ZB. A frequently unrecognized entity with emphasis on a plexiform variant. Adv Clin Pathol. 2000;4:25–33.

    CAS  Google Scholar 

  29. Mentzel T, Kutzner H. Reticular and plexiform perineurioma: clinicopathological and immunohistochemical analysis of two cases and review of perineurial neoplasms of skin and soft tissues. Virchows Arch. 2005;447:677–82.

    Article  PubMed  Google Scholar 

  30. Kawakami F, Hirose T, Kimoto A, Komori T, Itoh T. Plexiform perineurioma of the lip: a cases report and review of the literature. Pathol Int. 2012;62:704–8.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors are indebted to Ms. BreAnne MacKenzie and Mr. Brock Tidstrom (University of Minnesota) for their invaluable assistance in identifying the archived histologic slides and paraffin blocks, and tabulating the data of the oral peripheral nerve sheath tumor study, to Mr. Jonathan Henriksen (University of Minnesota) for his superb assistance with the illustrations, and Mrs. Denise Chase (Mayo Clinic), for her secretarial assistance.

Author information

Authors and Affiliations

  1. Division of Oral and Maxillofacial Pathology, School of Dentistry, University of Minnesota, 515 Delaware Street SE #16-206B, Minneapolis, MN, 55455, USA

    Ioannis G. Koutlas

  2. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA

    Bernd W. Scheithauer

Authors
  1. Ioannis G. Koutlas
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Bernd W. Scheithauer
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ioannis G. Koutlas.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Koutlas, I.G., Scheithauer, B.W. On Pseudo-Onion Bulb Intraneural Proliferations of the Non-Major Nerves of the Oral Mucosa. Head and Neck Pathol 7, 334–343 (2013). https://doi.org/10.1007/s12105-013-0446-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12105-013-0446-z

Keywords

Search

Navigation