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Extraneural Sclerosing Perineurioma of the Buccal Mucosa: A Case Report and Clinicopathologic Review

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Abstract

The perineurioma is an infrequently encountered benign peripheral nerve sheath tumor composed of a clonal proliferation of perineurial cells. Rare cases of perineurioma have been reported in the oral cavity. An extraneural sclerosing perineurioma arising in the buccal mucosa of a 17-year-old male is presented. Histopathologically, the tumor is composed of a well circumscribed nodular proliferation of spindle cells arranged in a storiform growth pattern, in some areas subtly arranged around vascular channels. The tumor cells reveal positive immunostaining for epithelial membrane antigen (EMA), collagen type IV and vimentin, and negative immunostaining for S-100 protein, consistent with a perineurial origin. To the best of our knowledge, this case represents the first report of an extraneural sclerosing perineurioma involving the oral cavity.

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References

  1. Lazarus SS, Trombetta LD. Ultrastructural identification of a benign perineurial cell tumor. Cancer. 1978;41(5):1823–9.

    Article  CAS  PubMed  Google Scholar 

  2. Fetsch JF, Miettinen M. Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a predilection for the fingers and palms of young adults. Am J Surg Pathol. 1997;21(12):1433–42.

    Article  CAS  PubMed  Google Scholar 

  3. Barrett AW, Hopper C, Landon G. Intra-osseous soft tissue perineurioma of the inferior alveolar nerve. Oral Oncol. 2002;38(8):793–6.

    Article  CAS  PubMed  Google Scholar 

  4. 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 

  5. 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 

  6. 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 

  7. 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 

  8. Graadt van Roggen JF, McMenamin ME, Belchis DA, Nielsen GP, Rosenberg AE, Fletcher CD. Reticular perineurioma: a distinctive variant of soft tissue perineurioma. Am J Surg Pathol. 2001;25(4):485–93.

    Article  CAS  PubMed  Google Scholar 

  9. Hornick JL, Fletcher CD. Soft tissue perineurioma: clinicopathologic analysis of 81 cases including those with atypical histologic features. Am J Surg Pathol. 2005;29(7):845–58.

    Article  PubMed  Google Scholar 

  10. Huguet P, De la Torre J, Pallares J, Carrera M, Soler F, Espinet B, 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 

  11. Ide F, Shimoyama T, Horie N, Kusama K. Comparative ultrastructural and immunohistochemical study of perineurioma and neurofibroma of the oral mucosa. Oral Oncol. 2004;40(9):948–53.

    Article  CAS  PubMed  Google Scholar 

  12. Kusama K, Iwamoto A, Mikuni M, Komagamine M, Suzuki T, Yamamura J, et al. A case of central perineurioma (Lazarus and Trombetta) of the mandible. J Nihon Univ Sch Dent. 1981;23(1):10–7.

    CAS  PubMed  Google Scholar 

  13. Meer S, Coleman H, Altini M. Intraoral perineurioma: report of a case with a review of the literature. Oral Dis. 2003;9(2):99–103.

    Article  CAS  PubMed  Google Scholar 

  14. 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(4):677–82.

    Article  PubMed  Google Scholar 

  15. 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 

  16. Tanaka A, Alva PG, Miyazaki Y, Yoshida N, Kaneko T, Oku Y, et al. Intraneural perineurioma of the tongue: report of a case and review of the literature. Oral Med Pathol. 2009;13:71–4.

    Article  Google Scholar 

  17. Shanthaveerappa TR, Bourne GH. Perineural epithelium: a new concept of its role in the integrity of the peripheral nervous system. Science. 1966;154(755):1464–7.

    Article  CAS  PubMed  Google Scholar 

  18. Giannini C, Scheithauer BW, Jenkins RB, Erlandson RA, Perry A, Borell TJ, 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(2):164–73.

    Article  CAS  PubMed  Google Scholar 

  19. 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.

    CAS  PubMed  Google Scholar 

  20. Lasota J, Wozniak A, Debiec-Rychter M. Loss of chromosome 22q and lack of NF2 mutations in perineuriomas [abstract 46]. Mod Pathol. 2000;13(11a).

  21. Pitchford CW, Schwartz HS, Atkinson JB, Cates JM. Soft tissue perineurioma in a patient with neurofibromatosis type 2: a tumor not previously associated with the NF2 syndrome. Am J Surg Pathol. 2006;30(12):1624–9.

    Article  PubMed  Google Scholar 

  22. Ausmus GG, Piliang MP, Bergfeld WF, Goldblum JR. Soft-tissue perineurioma in a 20-year-old patient with neurofibromatosis type 1 (NF1): report of a case and review of the literature. J Cutan Pathol. 2007;34(9):726–30.

    Article  PubMed  Google Scholar 

  23. Sciot R, Dal Cin P, Hagemeijer A, De Smet L, Van Damme B, Van den Berghe H. Cutaneous sclerosing perineurioma with cryptic NF2 gene deletion. Am J Surg Pathol. 1999;23(7):849–53.

    Article  CAS  PubMed  Google Scholar 

  24. Hahn H, Fletcher C. The role of cytogenetics and molecular genetics in soft tissue tumour diagnosis–a realistic appraisal. Curr Diagn Pathol. 2005;11:361–70.

    Article  Google Scholar 

  25. Brock JE, Perez-Atayde AR, Kozakewich HP, Richkind KE, Fletcher JA, Vargas SO. Cytogenetic aberrations in perineurioma: variation with subtype. Am J Surg Pathol. 2005;29(9):1164–9.

    Article  PubMed  Google Scholar 

  26. Mentzel T, Dei Tos AP, Fletcher CD. Perineurioma (storiform perineurial fibroma): clinico-pathological analysis of four cases. Histopathology. 1994;25(3):261–7.

    Article  CAS  PubMed  Google Scholar 

  27. Robson AM, Calonje E. Cutaneous perineurioma: a poorly recognized tumour often misdiagnosed as epithelioid histiocytoma. Histopathology. 2000;37(4):332–9.

    Article  CAS  PubMed  Google Scholar 

  28. Lopez JI, Elizalde JM. A case of perineurioma with prominent myxoid changes. Arch Anat Cytol Pathol. 1992;40(4):220–2.

    CAS  PubMed  Google Scholar 

  29. Macarenco RS, Ellinger F, Oliveira AM. Perineurioma: a distinctive and underrecognized peripheral nerve sheath neoplasm. Arch Pathol Lab Med. 2007;131(4):625–36.

    PubMed  Google Scholar 

  30. Zarineh A, Costa ME, Rabkin MS. Multiple hybrid granular cell tumor-perineuriomas. Am J Surg Pathol. 2008;32(10):1572–7.

    Article  PubMed  Google Scholar 

  31. Tsang WY, Chan JK, Chow LT, Tse CC. Perineurioma: an uncommon soft tissue neoplasm distinct from localized hypertrophic neuropathy and neurofibroma. Am J Surg Pathol. 1992;16(8):756–63.

    Article  CAS  PubMed  Google Scholar 

  32. Rank JP, Rostad SW. Perineurioma with ossification: a case report with immunohistochemical and ultrastructural studies. Arch Pathol Lab Med. 1998;122(4):366–70.

    CAS  PubMed  Google Scholar 

  33. Diaz-Flores L, Alvarez-Arguelles H, Madrid JF, Varela H, Gonzalez MP, Gutierrez R. Perineurial cell tumor (perineurioma) with granular cells. J Cutan Pathol. 1997;24(9):575–9.

    Article  CAS  PubMed  Google Scholar 

  34. Ariza A, Bilbao JM, Rosai J. Immunohistochemical detection of epithelial membrane antigen in normal perineurial cells and perineurioma. Am J Surg Pathol. 1988;12(9):678–83.

    Article  CAS  PubMed  Google Scholar 

  35. Theaker JM, Fletcher CD. Epithelial membrane antigen expression by the perineurial cell: further studies of peripheral nerve lesions. Histopathology. 1989;14(6):581–92.

    Article  CAS  PubMed  Google Scholar 

  36. Theaker JM, Gatter KC, Puddle J. Epithelial membrane antigen expression by the perineurium of peripheral nerve and in peripheral nerve tumours. Histopathology. 1988;13(2):171–9.

    Article  CAS  PubMed  Google Scholar 

  37. Weidenheim KM, Campbell WG Jr. Perineural cell tumor. Immunocytochemical and ultrastructural characterization. Relationship to other peripheral nerve tumors with a review of the literature. Virchows Arch A Pathol Anat Histopathol. 1986;408(4):375–83.

    Article  CAS  PubMed  Google Scholar 

  38. Hirose T, Scheithauer BW, Sano T. Perineurial malignant peripheral nerve sheath tumor (MPNST): a clinicopathologic, immunohistochemical, and ultrastructural study of seven cases. Am J Surg Pathol. 1998;22(11):1368–78.

    Article  CAS  PubMed  Google Scholar 

  39. Rosenberg AS, Langee CL, Stevens GL, Morgan MB. Malignant peripheral nerve sheath tumor with perineurial differentiation: “malignant perineurioma”. J Cutan Pathol. 2002;29(6):362–7.

    Article  PubMed  Google Scholar 

  40. Karaki S, Mochida J, Lee YH, Nishimura K, Tsutsumi Y. Low-grade malignant perineurioma of the paravertebral column, transforming into a high-grade malignancy. Pathol Int. 1999;49(9):820–5.

    Article  CAS  PubMed  Google Scholar 

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Acknowledgments

We acknowledge Dr. Christopher D.M. Fletcher, professor of pathology and director of surgical pathology, Brigham and Women’s Hospital, Boston, MA for his assistance in the histopathologic evaluation of this case. Additionally, we thank Ms. Anita Knighton and the laboratory staff in the Department of Pathology and Laboratory Medicine at Harvard Vanguard Medical Associates for technical expertise and Dr. George Gallagher and Dr. Devaki Sundararajan, Boston University School of Dental Medicine, for assistance with photomicroscopy.

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Authors and Affiliations

  1. Department of Pathology, Harvard Vanguard Medical Associates, 133 Brookline Ave., 6th Floor, Boston, MA, 02215, USA

    Vikki L. Noonan, Gilbert Brodsky & Sadru P. Kabani

  2. Oral and Maxillofacial Surgery, Private Practice, Nashua, NH, USA

    David J. Greene

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  1. Vikki L. Noonan
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Correspondence to Vikki L. Noonan.

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Noonan, V.L., Greene, D.J., Brodsky, G. et al. Extraneural Sclerosing Perineurioma of the Buccal Mucosa: A Case Report and Clinicopathologic Review. Head and Neck Pathol 4, 169–173 (2010). https://doi.org/10.1007/s12105-010-0175-5

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  • DOI: https://doi.org/10.1007/s12105-010-0175-5

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