Head and Neck Pathology

, Volume 12, Issue 1, pp 75–81 | Cite as

Multiple Desmoplastic Cellular Neurothekeomas in Child: Report of the First Oral Case and Review of the Literature

  • Tatiana Fernandes Araújo Almeida
  • Flaviana Dornela Verli
  • Cássio Roberto Rocha dos Santos
  • Saulo Gabriel Moreira Falci
  • Luciana Yamamoto Almeida
  • Lana Kei Yamamoto Almeida
  • Ana Terezinha Marques Mesquita
  • Jorge Esquiche León
Original Paper


Cellular neurothekeomas (CNs) are distinctive benign tumors of uncertain histogenesis, with predilection for the skin of the head and neck region. We describe the first case of multiple desmoplastic CNs (DCNs) affecting the oral cavity in a 9-year-old girl. Histopathologic evaluation showed a proliferation of spindle and epithelioid cells, forming nests and bundles, supported by exuberant fibrous stroma, as well as scattered multinucleated floret-like giant cells. The tumor cells were immunopositive for vimentin, CD63, CD56, whereas AE1/AE3, S100, CD34, α-SMA, GFAP, EMA, CD57 and NSE were negative. Ki-67 was <2%. Multiple DCNs should be considered in the differential diagnosis of oral nodular lesions.


Oral cavity Cellular neurothekeoma Desmoplastic cellular neurothekeoma Immunohistochemistry Child 



The authors would like to thank the parents and child who agreed to participate in this study.


  1. 1.
    Hornick JL, Fletcher CD. Cellular neurothekeoma: detailed characterization in a series of 133 cases. Am J Surg Pathol. 2007;31(3):329–40.CrossRefPubMedGoogle Scholar
  2. 2.
    Fetsch JF, Laskin WB, Miettinen M. Nerve sheath myxoma: a clinicopathologic and immunohistochemical analysis of 57 morphologically distinctive, S-100 protein- and GFAP-positive, myxoid peripheral nerve sheath tumors with a predilection for the extremities and a high local recurrence rate. Am J Surg Pathol. 2005;29(12):1615–24.CrossRefPubMedGoogle Scholar
  3. 3.
    Vered M, Fridman E, Carpenter WM, Buchner A. Classic neurothekeoma (nerve sheath myxoma) and cellular neurothekeoma of the oral mucosa: immunohistochemical profiles. J Oral Pathol Med. 2011;40(2):174–80.CrossRefPubMedGoogle Scholar
  4. 4.
    Fetsch JF, Laskin WB, Hallman JR, Lupton GP, Miettinen M. Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information. Am J Surg Pathol. 2007;31(7):1103–114.CrossRefPubMedGoogle Scholar
  5. 5.
    Zedek DC, White WL, McCalmont TH. Desmoplastic cellular neurothekeoma: clinicopathological analysis of twelve cases. J Cutan Pathol. 2009;36(11):1185–90.CrossRefPubMedGoogle Scholar
  6. 6.
    Barnhill RL, Mihm MC Jr. Cellular neurothekeoma. A distinctive variant of neurothekeoma mimicking nevomelanocytic tumors. Am J Surg Pathol. 1990;14(2):113–20.CrossRefPubMedGoogle Scholar
  7. 7.
    D’Antonio A, Cuomo R, Angrisani B, Memoli D, Angrisani P. Desmoplastic cellular neurothekeoma mimicking a desmoplastic melanocytic tumor. Am Acad Dermatol. 2011;65(2):e57–e8.CrossRefGoogle Scholar
  8. 8.
    Weng YC, Shen JL, Yang CS, Li WH. Desmoplastic cellular neurothekeoma mimicking basalcell carcinoma. Dtsch Dermatol Ges. 2015;13(3):243–5.Google Scholar
  9. 9.
    Mahalingam M, Alter JN, Bhawan J. Multiple cellular neurothekeomas—a case report and review on the role of immunohistochemistry as a histologic adjunct. J Cutan Pathol. 2006;33(11):51–6.CrossRefPubMedGoogle Scholar
  10. 10.
    Garcia-Gutierrez M, Toussaint-Caire S, Gonzalez-Sanchez P, Ortiz-Hidalgo C. Multiple desmoplastic cellular neurothekeomas localized to the face of a 16-year-old boy. Am J Dermatopathol. 2010;32(8):509–13.CrossRefPubMedGoogle Scholar
  11. 11.
    Gallager RL, Helwig EB. Neurothekeoma—a benign cutaneous tumor of neural origin. Am J Clin Pathol. 1980;74(6):759–64.CrossRefPubMedGoogle Scholar
  12. 12.
    Rosati LA, Fratamico FC, Eusebi V. Cellular neurothekeoma. Appl Pathol. 1986;4(3):186–91.PubMedGoogle Scholar
  13. 13.
    Husain S, Silvers DN, Halperin AJ, McNutt NS. Histologic spectrum of neurothekeoma and the value of immunoperoxidase staining for S-100 protein in distinguishing it from melanoma. Am J Dermatopathol. 1994;16(5):496–503.CrossRefPubMedGoogle Scholar
  14. 14.
    Barnhill RL, Dickersin GR, Nickeleit V, Bhan AK, Muhlbauer JE, Phillips ME, et al. Studies on the cellular origin of neurothekeoma: clinical, light microscopic, immunohistochemical, and ultrastructural observations. Am Acad Dermatol. 1991;25(1 ):80–8.CrossRefGoogle Scholar
  15. 15.
    Laskin WB, Fetsch JF, Miettinen M. ‎The “neurothekeoma”: immunohistochemical analysis distinguishes the true nerve sheath myxoma from its mimics. ‎Hum Pathol. 2000;31(10):1230–41.CrossRefPubMedGoogle Scholar
  16. 16.
    Barrett AW, Suhr M. Cellular neurothekeoma of the oral mucosa. Oral Oncol. 2001;37(8):660–4.CrossRefPubMedGoogle Scholar
  17. 17.
    Nishioka M, Aguirre RL, Ishikawa A, Nagumo K, Wang LH, Okada N. Nerve sheath myxoma (neurothekeoma) arising in the oral cavity: histological and immunohistochemical features of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontology. 2009;107(5):e28–e33.CrossRefGoogle Scholar
  18. 18.
    Prado JD, Andrade RG, Silva-Sousa YT, Andrade MF, Soares FA, Perez DE. Nerve sheath myxoma of the gingiva: report of a rare case and review of the literature. J Periodontol. 2007;78(8):1639–43.CrossRefPubMedGoogle Scholar
  19. 19.
    Rodriguez-Peralto JL, el-Naggar AK. Neurothekeoma of the oral cavity: case report and review of the literature. ‎‎J Oral Maxillofac Surg. 1992;50(11):1224–6.CrossRefPubMedGoogle Scholar
  20. 20.
    Rozza-de-Menezes RE, Andrade RM, Israel MS, Goncalves Cunha KS. Intraoral nerve sheath myxoma: case report and systematic review of the literature. Head Neck. 2013;35(12):E397–E404.CrossRefPubMedGoogle Scholar
  21. 21.
    Sist TC Jr, Greene GW Jr. Benign nerve sheath myxoma: light and electron microscopic features of two cases. Oral Surg Oral Med Oral Pathol. 1979;47(5):441–4.CrossRefPubMedGoogle Scholar
  22. 22.
    Tiffee JC, Pulitzer DR. Nerve sheath myxoma of the oral cavity: case report and review. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontology. 1996;82(4):423–5.CrossRefGoogle Scholar
  23. 23.
    Spadari F, Guzzi G, Bombeccari GP, Mariani U, Gianatti A, Ruffoni D, et al. Nerve sheath myxoma of the tongue. Acta Dermatovenerol Croat. 2014;22(1):52–6.PubMedGoogle Scholar
  24. 24.
    Breuer T, Koester M, Weidenbecher M, Steininger H. Neurothekeoma, a rare tumour of the tongue. J Otorhinolaryngol Relat Spec. 1999;61(3):161–4.CrossRefGoogle Scholar
  25. 25.
    Penarrocha M, Bonet J, Minguez JM, Vera F. Nerve sheath myxoma (neurothekeoma) in the tongue of a newborn. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontology. 2000;90(1):74–7.CrossRefGoogle Scholar
  26. 26.
    Kim HJ, Baek CH, Ko YH, Choi JY. Neurothekeoma of the tongue: CT, MR, and FDG PET imaging findings. Am J Neuroradiol. 2006;27(9):1823–5.PubMedGoogle Scholar
  27. 27.
    Plaza JA, Torres-Cabala C, Evans H, Diwan AH, Prieto VG. Immunohistochemical expression of S100A6 in cellular neurothekeoma: clinicopathologic and immunohistochemical analysis of 31 cases. Am J Dermatopathol. 2009;31(5):419–22.CrossRefPubMedGoogle Scholar
  28. 28.
    Safadi RA, Hellstein JW, Diab MM, Hammad HM. Nerve sheath myxoma (neurothekeoma) of the gingiva, a case report and review of the literature. Head Neck Pathol. 2010;4(3):242–5.CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Pan HY, Tseng SH, Weng CC, Chen Y. Cellular neurothekeoma of the upper lip in an infant. Pediatr Neonatol. 2014;55(1):71–4.CrossRefPubMedGoogle Scholar
  30. 30.
    Emami N, Zawawi F, Ywakim R, Nahal A, Daniel SJ. Oral cellular neurothekeoma. Case Rep Otolaryngol. 2013;2013:935435.PubMedPubMedCentralGoogle Scholar
  31. 31.
    Ishikawa M, Yamamoto T, Izumi M. Cellular Neurothekeoma on the Lip. ‎Indian J Dermatol. 2016;61(1):122.CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Gonzalez-Arriagada WA, Leon JE, Vargas PA, Paes de Almeida O, Lopes MA. Intraoral sclerosing perineurioma: a case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontology. 2010;109(5):e46–e52.CrossRefGoogle Scholar
  33. 33.
    Magro G, Amico P, Vecchio GM, Caltabiano R, Castaing M, Kacerovska D, et al. Multinucleated floret-like giant cells in sporadic and NF1-associated neurofibromas: a clinicopathologic study of 94 cases. Virchows Arch. 2010;456(1):71–6.CrossRefPubMedGoogle Scholar
  34. 34.
    de Andrade CR, Lopes MA, de Almeida OP, Leon JE, Mistro F, Kignel S. Giant cell angiofibroma of the oral cavity: a case report and review of the literature. Med Oral Patol Oral Cir Bucal. 2008;13(9):E540–E3.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  • Tatiana Fernandes Araújo Almeida
    • 1
  • Flaviana Dornela Verli
    • 2
  • Cássio Roberto Rocha dos Santos
    • 1
  • Saulo Gabriel Moreira Falci
    • 1
  • Luciana Yamamoto Almeida
    • 3
  • Lana Kei Yamamoto Almeida
    • 4
  • Ana Terezinha Marques Mesquita
    • 1
  • Jorge Esquiche León
    • 5
  1. 1.Department of Dentistry, Stomatology ClinicalFederal University of Vales do Jequitinhonha e Mucuri (UFVJM) DiamantinaBrazil
  2. 2.Laboratory of Pathology, Department of Basic SciencesFederal University of Vales do Jequitinhonha e Mucuri (UFVJM)DiamantinaBrazil
  3. 3.Hematology Division, Department of Clinical Medicine, Medical School of Ribeirão PretoUniversity of São Paulo (FMRP/USP)Ribeirão PretoBrazil
  4. 4.Department of Pediatric Dentistry, Araraquara Dental SchoolUniversidade Estadual Paulista-UNESPAraraquaraBrazil
  5. 5.Oral Pathology, Department of Stomatology, Public Oral Health and Forensic Dentistry, School of Dentistry of Ribeirão PretoUniversity of São Paulo (FORP/USP)Ribeirão PretoBrazil

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