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Sinonasal Tract Neurofibroma: A Clinicopathologic Series of 12 Cases with a Review of the Literature

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Abstract

Neurofibroma (NF), a benign peripheral nerve sheath tumor, is very uncommon in the sinonasal tract, with only a few reported cases in the English literature. Cases within the files of the authors’ institutions confined to the sinonasal tract were compared to cases reported in the English literature (Medline 1966–2014). The 12 patients included 6 females and 6 males, aged 26–75 years (mean 46.2 years). The patients usually presented clinically with a mass lesion (n = 11), obstruction (n = 4) or pain (n = 3), with an average symptom duration of 42.9 months. Two patients had neurofibromatosis (NF1). Tumors involved the nasal cavity alone (n = 8), maxillary sinus alone (n = 2), or mixed sites (n = 2), with a range of 0.4–4.1 cm (mean 2.2 cm). The tumors were circumscribed, composed of spindled to wavy cells with curvilinear nuclei set in a background of collagenized stroma and mast cells. Nuclear palisading and perivascular hyalinization were not seen. Mitoses were scant. Pleomorphism, necrosis and increased cellularity were absent. By immunohistochemistry, the lesional cells were S100 protein, SOX10 and NFP positive, while CD34 highlighted the perineurium. INI1 was intact, with strong nuclear expression in all cases. All patients had surgical excision without recurrence (mean follow-up 8.6 years). The principle differential diagnoses include schwannoma, perineurioma, fibromatosis, and solitary fibrous tumor. NF of the sinonasal tract occurs in middle-aged patients without a gender predilection, usually with non-specific symptoms present for a long duration. Tumors are relatively large (mean 2.2), and usually affect one site only. Surgery is curative, with only 16.7 % NF1 associated. S100 protein, SOX10 and NFP highlight the Schwann cells, with CD34 highlighting the perineural fibroblasts.

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References

  1. Agarwal MK, Gupta OP, Samant HC, et al. Neurofibroma of the maxillary antrum. Oral Surg Oral Med Oral Pathol. 1979;48:150–2.

    Article  CAS  PubMed  Google Scholar 

  2. Annino D J Jr, Domanowski GF, Vaughan CW. A rare cause of nasal obstruction: a solitary neurofibroma. Otolaryngol Head Neck Surg. 1991;104:484–8.

    PubMed  Google Scholar 

  3. Biswas D, Mal R. Bilateral solitary maxillary sinus neurofibroma. Ear Nose Throat J. 2010;89:E1–2.

    PubMed  Google Scholar 

  4. Bradley PJ, Singh SD. Congenital nasal masses: diagnosis and management. Clin Otolaryngol Allied Sci. 1982;7:87–97.

    Article  CAS  PubMed  Google Scholar 

  5. Hillstrom RP, Zarbo RJ, Jacobs JR. Nerve sheath tumors of the paranasal sinuses: electron microscopy and histopathologic diagnosis. Otolaryngol Head Neck Surg. 1990;102:257–63.

    CAS  PubMed  Google Scholar 

  6. Kim YD, Bai CH, Suh JS, et al. Transnasal endoscopic excision of an isolated neurofibroma of the nasal septum. Rhinology. 1997;35:89–91.

    CAS  PubMed  Google Scholar 

  7. Kohli PS, Goel K. Endoscopic excision of solitary neurofibroma arising from posterior nasal septum. Clin Rhinol. 2011;4:43–6.

    Article  Google Scholar 

  8. Kuroda N, Kazakov DV, Hes O, et al. Hybrid peripheral nerve sheath tumor of the nasal cavity showing schwannomatous, neurofibromatous, and perineuriomatous areas. Med Mol Morphol. 2010;43:82–5.

    Article  PubMed  Google Scholar 

  9. Manganaris A, Tsompanidou C, Manganaris T. A peripheral nerve sheath tumour as a cause of nasal obstruction. J Laryngol Otol. 2006;120:e44.

    Article  CAS  PubMed  Google Scholar 

  10. Mori H, Kakuta S, Yamaguchi A, et al. Solitary intraosseous neurofibroma of the maxilla: report of a case. J Oral Maxillofac Surg. 1993;51:688–90.

    Article  CAS  PubMed  Google Scholar 

  11. Perzin KH, Panyu H, Wechter S. Nonepithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx. A clinicopathologic study. XII: schwann cell tumors (neurilemoma, neurofibroma, malignant schwannoma). Cancer. 1982;50:2193–202.

    Article  CAS  PubMed  Google Scholar 

  12. Robitaille Y, Seemayer TA, El DA. Peripheral nerve tumors involving paranasal sinuses: a case report and review of the literature. Cancer. 1975;35:1254–8.

    Article  CAS  PubMed  Google Scholar 

  13. Stevens DJ, Kirkham N. Neurofibromas of the paranasal sinuses. J Laryngol Otol. 1988;102:256–9.

    Article  CAS  PubMed  Google Scholar 

  14. Vaideeswar P, Madiwale CV, Kathpal D, et al. Sinonasal neurogenic tumours. Indian J Pathol Microbiol. 2002;45:161–3.

    PubMed  Google Scholar 

  15. Chakravarti A, Vishwakarma SK, Arora VK. Solitary neurofibroma causing nasal obstruction. J Indian Med Assoc. 1999;97:526.

    CAS  PubMed  Google Scholar 

  16. Chitale AR, Rege SR, Karnik PP, et al. Uncommon polypoid tumours of nasal cavity. Indian J Cancer. 1974;11:244–54.

    CAS  PubMed  Google Scholar 

  17. Chua CN, Alhady M, Ngo CT, et al. Solitary nasal neurofibroma presenting as compressive optic neuropathy. Eye (Lond). 2006;20:1406–8.

    Article  CAS  Google Scholar 

  18. Heffner DK. Problems in pediatric otorhinolaryngic pathology, III. Teratoid and neural tumors of the nose, sinonasal tract, and nasopharynx. Int J Pediatr Otorhinolaryngol. 1983;6:1–21.

    Article  CAS  PubMed  Google Scholar 

  19. Hellquist HB, Lundgren J. Neurogenic sarcoma of the sinonasal tract. J Laryngol Otol. 1991;105:186–90.

    Article  CAS  PubMed  Google Scholar 

  20. High AS, Kay NJ. Solitary neurogenic sarcoma of the nose. J Laryngol Otol. 1985;99:1151–9.

    Article  CAS  PubMed  Google Scholar 

  21. Hirao M, Gushiken T, Imokawa H, et al. Solitary neurofibroma of the nasal cavity: resection with endoscopic surgery. J Laryngol Otol. 2001;115:1012–4.

    Article  CAS  PubMed  Google Scholar 

  22. Holsinger FC, Hafemeister AC, Hicks MJ, et al. Differential diagnosis of pediatric tumors of the nasal cavity and paranasal sinuses: a 45-year multi-institutional review. Ear Nose Throat J. 2010;89:534–40.

    PubMed  Google Scholar 

  23. Ijaduola TG, Ademiluyi SA. Otorhinolaryngological manifestations of von Recklinghausen’s disease in Nigerians. J Laryngol Otol. 1986;100:115–23.

    Article  CAS  PubMed  Google Scholar 

  24. Kimberling WJ, Lynch HT, Grush ML. Concurrent von Recklinghausen’s neurofibromatosis, hereditary spherocytosis and fronto nasal dysplasia. Nebr Med J. 1982;67:151–4.

    CAS  PubMed  Google Scholar 

  25. Knight W A III, Murphy WK, Gottlieb JA. Neurofibromatosis associated with malignant neurofibromas. Arch Dermatol. 1973;107:747–50.

    Article  PubMed  Google Scholar 

  26. Krammer U, Wimmer K, Wiesbauer P, et al. Neurofibromatosis 1: a novel NF1 mutation in an 11-year-old girl with a giant cell granuloma. J Child Neurol. 2003;18:371–3.

    Article  PubMed  Google Scholar 

  27. Lee JH, Bae JH, Kim KS. A case of solitary neurofibroma of the nasal dorsum: resection using an external rhinoplasty approach. Eur Arch Otorhinolaryngol. 2005;262:813–5.

    Article  PubMed  Google Scholar 

  28. Macomber WB, Wang MK. Congenital neoplasms of the nose. Plast Reconstr Surg. 1946;1953(11):215–29.

    Google Scholar 

  29. Mondal AR, Rashid MA, Bera SP, et al. Neurofibroma of paranasal sinuses—a case report. Indian J Otolaryngol Head Neck Surg. 2004;56:40–2.

    PubMed Central  CAS  PubMed  Google Scholar 

  30. Moreno PM, Meseguer DH. Solitary neurofibroma of the inferior nasal turbinate. Auris Nasus Larynx. 1998;25:329–31.

    Article  CAS  PubMed  Google Scholar 

  31. Oi H, Watanabe Y, Shojaku H, et al. Nasal Septal Neurinoma Acta Otolaryngol Suppl. 1993;504:151–4.

    Article  CAS  PubMed  Google Scholar 

  32. Pantazopoulos PE. Schwannomas of nose, oral cavity and pharynx. Acta Otolaryngol. 1965;60:97–104.

    Article  CAS  PubMed  Google Scholar 

  33. Pasic TR, Makielski K. Nasal schwannoma. Otolaryngol Head Neck Surg. 1990;103:943–6.

    CAS  PubMed  Google Scholar 

  34. Putney FJ, Moran JJ, Thomas GK. Neurogenic tumors of the head and neck. Laryngoscope. 1964;74:1037–59.

    CAS  PubMed  Google Scholar 

  35. Rakshit BK, Sundareshwar B, Reddy CR. Nerve sheath tumors. Int Surg. 1973;58:557–60.

    CAS  PubMed  Google Scholar 

  36. Rengahary SS, McMahon M, Bigongiari LR, et al. Neurofibroma of the infratemporal fossa: case report and technical note. Neurosurgery. 1982;11:43–7.

    Article  CAS  PubMed  Google Scholar 

  37. Sharma DK, Sohal BS, Parmar TL, et al. Schwannomas of head and neck and review of literature. Indian J Otolaryngol Head Neck Surg. 2012;64:177–80.

    Article  PubMed Central  PubMed  Google Scholar 

  38. Sharma SB, Hong P. Solitary neurofibroma of the frontal sinus. Case Rep Otolaryngol. 2012;2012:373808.

    PubMed Central  PubMed  Google Scholar 

  39. Shugar JM, Som PM, Biller HF, et al. Peripheral nerve sheath tumors of the paranasal sinuses. Head Neck Surg. 1981;4:72–6.

    Article  CAS  PubMed  Google Scholar 

  40. Sinha R, Basu S, Ganguly S, et al. Trilobed intranasal neurofibroma—an unusual presentation. J Indian Med Assoc. 1997;95(192):196.

    Google Scholar 

  41. Vujevich JJ, Goldberg LH, Kimyai-Asadi A, et al. Recurrent nodule on the nasal columella: a good reason to re-biopsy. Int J Dermatol. 2008;47:728–31.

    Article  PubMed  Google Scholar 

  42. Wilson JA, McLaren K, McIntyre MA, et al. Nerve-sheath tumors of the head and neck. Ear Nose Throat J. 1988;67(103–7):110.

    Google Scholar 

  43. Cunha KS, Caruso AC, Faria PA, et al. Evaluation of Bcl-2, Bcl-x and cleaved caspase-3 in malignant peripheral nerve sheath tumors and neurofibromas. An Acad Bras Cienc. 2013;85:1497–511.

    Article  PubMed  Google Scholar 

  44. Fine SW, McClain SA, Li M. Immunohistochemical staining for calretinin is useful for differentiating schwannomas from neurofibromas. Am J Clin Pathol. 2004;122:552–9.

    Article  PubMed  Google Scholar 

  45. Ohashi R, Wakayama N, Kawamoto M, et al. Solitary nasal schwannoma: usefulness of CD34 and calretinin staining for distinction from histological mimics. J Nippon Med Sch. 2013;80:300–6.

    Article  PubMed  Google Scholar 

  46. 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 

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

    Article  PubMed  Google Scholar 

  48. Luscan A, Shackleford G, Masliah-Planchon J, et al. The activation of the WNT signaling pathway is a Hallmark in neurofibromatosis type 1 tumorigenesis. Clin Cancer Res. 2014;20:358–71.

    Article  CAS  PubMed  Google Scholar 

  49. Lewis JT, Oliveira AM, Nascimento AG, et al. Low-grade sinonasal sarcoma with neural and myogenic features: a clinicopathologic analysis of 28 cases. Am J Surg Pathol. 2012;36:517–25.

    Article  PubMed  Google Scholar 

  50. Wenig BM. Recently described sinonasal tract lesions/neoplasms: considerations for the new world health organization book. Head Neck Pathol. 2014;8:33–41.

    Article  PubMed Central  PubMed  Google Scholar 

  51. Ferreiro JA, Nascimento AG. Solitary fibrous tumour of the major salivary glands. Histopathology. 1996;28:261–4.

    Article  CAS  PubMed  Google Scholar 

  52. Hanau CA, Miettinen M. Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. Hum Pathol. 1995;26:440–9.

    Article  CAS  PubMed  Google Scholar 

  53. Bauer JL, Miklos AZ, Thompson LD. Parotid gland solitary fibrous tumor: a case report and clinicopathologic review of 22 cases from the literature. Head Neck Pathol. 2012;6:21–31.

    Article  PubMed Central  PubMed  Google Scholar 

  54. Rudolph P, Schubert B, Wacker HH, et al. Immunophenotyping of dermal spindle cell tumors: diagnostic value of monocyte marker Ki-M1p and histogenetic considerations. Am J Surg Pathol. 1997;21:791–800.

    Article  CAS  PubMed  Google Scholar 

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Acknowledgments

The authors thank to Ms. Hannah B. Herrera for her conscientious research assistance.

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There is no financial conflict of interest.

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Authors and Affiliations

  1. Department of Pathology, Southern California Permanente Kaiser Medical Group, Woodland Hills Medical Center, 5601 De Soto Avenue, Woodland Hills, CA, 91365, USA

    Ari B. Azani & Lester D. R. Thompson

  2. Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA

    Justin A. Bishop

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Correspondence to Lester D. R. Thompson.

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The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Southern California Permanente Medical Group or The Permanente Medical Group.

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Azani, A.B., Bishop, J.A. & Thompson, L.D.R. Sinonasal Tract Neurofibroma: A Clinicopathologic Series of 12 Cases with a Review of the Literature. Head and Neck Pathol 9, 323–333 (2015). https://doi.org/10.1007/s12105-014-0593-x

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