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Intraoral Perineurioma, Soft Tissue Type: Report of Five Cases, Including 3 Intraosseous Examples, and Review of the Literature

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Abstract

Soft tissue type perineuriomas (STP) are uncommon tumors, oral examples being very rare. They have been described in the mandible, gingiva, lips, retrotonsillar mucosa and maxillary vestibule. Herein, we report the clinicopathologic features of five STP, two affecting the buccal mucosa and three the mandible. Three patients were women and two men. All tumors were characterized by a proliferation of cytologically bland, mitotically inactive spindled cells with round, ovoid or spindled nuclei, embedded in a variably collagenous and myxoid matrix. Interestingly, two mandibular tumors featured psammoma bodies and one, in addition, contained irregular calcifications. Tumor cells showed the immunohistochemical profile of perineurial cells including epithelial membrane antigen, Glut-1, claudin-1 and collagen type IV. S100 and neurofilament proteins were not expressed by the tumor cells. A few minute, peripherally situated, entrapped nerves were identified. All tumors were reported gross-totally excised and no recurrences have taken place. The clinical characteristics of STP are summarized and its differential diagnosis relative to other spindle cells tumors and meningioma is discussed.

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References

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

    Article  Google Scholar 

  2. Conley FK, Rubinstein LJ, Spence AM. Studies on experimental malignant nerve sheath tumors maintained in tissue and organ culture systems. II. Electron microscopy observations. Acta Neuropathol. 1976;34:293–310.

    Article  CAS  PubMed  Google Scholar 

  3. Bunge MB, Wood PM, Tynan LB, Bates ML, Sanes JR. Perineurium originates from fibroblasts: demonstration in vitro with a retroviral marker. Science. 1989;243:229–31.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  5. Erlandson RA. The enigmatic perineurial cell and its participation in tumors and in tumorlike entities. Ultrastruct Pathol. 1991;15:335–51.

    Article  CAS  PubMed  Google Scholar 

  6. Michal M, Kazakov DV, Belousova I, et al. A benign neoplasm with histopathological features of both schwannoma and retiform perineurioma (benign schwannoma-perineurioma): a report of six cases of a distinctive soft tissue tumor with a predilection for the fingers. Virchows Arch. 2004;445:347–53.

    Article  PubMed  Google Scholar 

  7. Hornick JL, Bundock EA, Fletcher CD. Hybrid schwannoma/perineurioma: clinicopathologic analysis of 42 distinctive benign nerve sheath tumors. Am J Surg Pathol 2009 (epub ahead of print).

  8. Zarineh A, Costa ME, Rabkin MS. Multiple hybrid granular cell tumors-perineuriomas. Am J Surg Pathol. 2008;32:1572–7.

    Article  PubMed  Google Scholar 

  9. Scheithauer BW, Woodruff J, Erlandson R. Tumors of peripheral nervous system. In: Rosai J, Sobin LH, editors. Atlas of tumor pathology. Washington, DC: Fascicle 24. Armed Forces Institute of Pathology; 1999. p. 177–218. Third Series.

    Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  12. 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;21:1433–42.

    Article  CAS  PubMed  Google Scholar 

  13. Graadt van Roggen JF, McMenamin ME, Belchis DA, et al. Reticular perineurioma: a distinctive variant of soft tissue perineurioma. Am J Surg Pathol. 2001;25:485–93.

    Article  CAS  PubMed  Google Scholar 

  14. Al-Daraji WI. Granular perineurioma: the first report of a rare distinctive subtype of perineurioma. Am J Dermatopathol. 2008;30:163–8.

    Article  PubMed  Google Scholar 

  15. Haider SA, Lemberger RJ, Fisher C, McCulloch. Epithelioid perineurioma: an unusual variant. J Clin Pathol. 2008;61:1130–2.

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  21. Morton Jr TH. Mandibular neoplasm (perineurioma). WESTOP Annual Meeting; 2007, Whistler, B.C., Canada.

  22. Landini G, Kitano M. Meningioma of the mandible. Cancer. 1992;69:2917–20.

    Article  CAS  PubMed  Google Scholar 

  23. Pinkus GS, Kurtin PJ. Epithelial membrane antigen—a diagnostic discriminant in surgical pathology: immunohistochemical profile in epithelial, mesenchymal, and hematopoietic neoplasms using paraffin sections and monoclonal antibodies. Hum Pathol. 1985;16:929–40.

    Article  CAS  PubMed  Google Scholar 

  24. Theaker JM, Gatter KC, Esiri MM, Fleming KA. Epithelial membrane antigen and cytokeratin expression by meningiomas: an immunohistological study. J Clin Pathol. 1986;39:435–9.

    Article  CAS  PubMed  Google Scholar 

  25. Theaker JM, Gillett MB, Fleming KA, Gatter KC. Epithelial membrane antigen expression by meningiomas, and the perineurium of peripheral nerve. Arch Pathol Lab Med. 1987;111:409.

    CAS  PubMed  Google Scholar 

  26. Lassmann H, Ammerer HP. Schwann cells and perineurium in neuroma. Some morphological aspects. Virchows Arch. 1974;15:313–21.

    CAS  Google Scholar 

  27. 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  CAS  PubMed  Google Scholar 

  28. Giannini C, Scheithauer BW, Jenkins RB, 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;21:164–73.

    Article  CAS  PubMed  Google Scholar 

  29. Lasota J, Fetch 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.

    CAS  PubMed  Google Scholar 

  30. 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;22:1368–78.

    Article  CAS  PubMed  Google Scholar 

  31. Brock JE, Perez-Atayde AR, Kozakewich HP, et al. Cytogenetic aberrations in perineurioma: variation and subtype. Am J Surg Pathol. 2005;29:1164–9.

    Article  PubMed  Google Scholar 

  32. 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;16:756–63.

    Article  CAS  PubMed  Google Scholar 

  33. Rank JP, Rostad SW. Perineurioma with ossification. A case report with immunohistochemical and ultrastructural studies. Arch Pathol Lab Med. 1998;122:366–70.

    CAS  PubMed  Google Scholar 

  34. Jones AC, Freedman PD. Primary extracranial meningioma of the mandible. A report of 2 cases and a review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;91:338–41.

    Article  CAS  PubMed  Google Scholar 

  35. Lell M, Tudor C, Aigner T, Kessler P. Primary intraosseous meningioma of the mandible: CT and MR imaging features. Am J Neuroradiol. 2007;28:129–31.

    Article  CAS  PubMed  Google Scholar 

  36. Yamaguchi U, Hasegawa T, Hirose T, et al. Sclerosing perineurioma: a clinicopathological study of five cases and diagnostic utility of immunohistochemical staining for GLUT1. Virch Arch. 2003;443:159–63.

    Article  Google Scholar 

  37. Smith ME, Awasthi R, O’Shaughnessy S, Fisher C. Evaluation of perineurial differentiation in epithelioid sarcoma. Histopathol. 2005;47:575–81.

    Article  CAS  Google Scholar 

  38. Folpe AL, Billings SD, McKenney JK, et al. Expression of claudin-1, a recently described tight junction-associated protein, distinguishes soft tissue perineurioma from potential mimics. Am J Surg Pathol. 2002;26:1620–6.

    Article  PubMed  Google Scholar 

  39. Hahn HP, Bundock EA, Hornick JL. Immunohistochemical staining for claudin-1 can help distinguish meningiomas from histologic mimics. Am J Clin Pathol. 2006;125:203–8.

    PubMed  Google Scholar 

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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.

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Authors and Affiliations

  1. Division of Oral and Maxillofacial Pathology, University of Minnesota, School of Dentistry, 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 & Andrew L. Folpe

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  1. Ioannis G. Koutlas
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  2. Bernd W. Scheithauer
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  3. Andrew L. Folpe
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Correspondence to Ioannis G. Koutlas.

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Koutlas, I.G., Scheithauer, B.W. & Folpe, A.L. Intraoral Perineurioma, Soft Tissue Type: Report of Five Cases, Including 3 Intraosseous Examples, and Review of the Literature. Head and Neck Pathol 4, 113–120 (2010). https://doi.org/10.1007/s12105-010-0177-3

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