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Nerve Sheath and Related Tumors

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Soft Tissue Tumors of the Skin

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Abstract

This chapter reviews the wide spectrum of cutaneous nerve sheath tumors. The vast majority are benign tumors, including neurofibromas, schwannomas, perineuriomas, granular cell tumors, and various neuroma subtypes. Malignant granular cell tumor, malignant peripheral nerve sheath tumor, and Ewing sarcoma, a malignant neuroectodermal tumor, are also rarely encountered in the skin. Diagnostic criteria and practical considerations are emphasized.

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Change history

  • 11 April 2019

    The original version of Chapter 10 was inadvertently published with one author name “Steven D. Billings” instead of two author names Youran Zou and Steven D. Billings. The chapter has been now updated.

Selected References

  1. Abbas O, Bhawan J. Cutaneous plexiform lesions. J Cutan Pathol. 2010;37(6):613–23.

    Article  PubMed  Google Scholar 

  2. Allison KH, Patel RM, Goldblum JR, Rubin BP. Superficial malignant peripheral nerve sheath tumor: a rare and challenging diagnosis. Am J Clin Pathol. 2005;124(5):685–92.

    Article  PubMed  Google Scholar 

  3. Avninder S, Ramesh V, Vermani S. Benign nerve sheath myxoma (myxoid neurothekeoma) in the leg. Dermatol Online J. 2007;13(2):14.

    PubMed  Google Scholar 

  4. Erstine EM, Ko JS, Rubin BP, McKenney JK, Goldblum JR, Billings SD. Broadening the anatomic landscape of sclerosing perineurioma: A series of 5 cases in nonacral sites. Am J Dermatopathol. 2016 Nov 11. [Epub ahead of print] PubMed.

    Google Scholar 

  5. Evans HL. Sporadic superficial diffuse neurofibromas with repeated local recurrence over many years and a tendency toward malignant change: a report of 3 cases. Am J Surg Pathol. 2013;37(7):987–94.

    Article  PubMed  Google Scholar 

  6. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. 1998;22(7):779–94.

    Article  CAS  PubMed  Google Scholar 

  7. Fletcher CD, Theaker JM. Digital pacinian neuroma: a distinctive hyperplastic lesion. Histopathology. 1989;15(3):249–56.

    Article  CAS  PubMed  Google Scholar 

  8. Fletcher CD, Davies SE, McKee PH. Cellular schwannoma: a distinct pseudosarcomatous entity. Histopathology. 1987;11(1):21–35.

    Article  CAS  PubMed  Google Scholar 

  9. Fox MD, Billings SD, Gleason BC, Thomas AB, Cibull TL. Cutaneous meningioma: a potential diagnostic pitfall in p63 positive cutaneous neoplasms. J Cutan Pathol. 2013;40(10):891–5.

    PubMed  Google Scholar 

  10. Goldblum JR, Beals TF, Weiss SW. Neuroblastoma-like neurilemoma. Am J Surg Pathol. 1994;18(3):266–73.

    Article  CAS  PubMed  Google Scholar 

  11. Hart J, Gardner JM, Edgar M, Weiss SW. Epithelioid schwannomas: an analysis of 58 cases including atypical variants. Am J Surg Pathol. 2016;40(5):704–13.

    Article  PubMed  Google Scholar 

  12. 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 

  13. Hornick JL, Bundock EA, Fletcher CD. Hybrid schwannoma/perineurioma: clinicopathologic analysis of 42 distinctive benign nerve sheath tumors. Am J Surg Pathol. 2009;33(10):1554–61.

    Article  PubMed  Google Scholar 

  14. Kacerovska D, Michal M, Kazakov DV. Hybrid Epithelioid Schwannoma/Perineurioma. Am J Dermatopathol. 2016;38(7):e90–2.

    Article  PubMed  Google Scholar 

  15. Kaku Y, Fukumoto T, Louise Opada G, Anan T, Kimura T. A clinicopathologic and immunohistochemical study of 7 cases of sclerosing perineurioma. Am J Dermatopathol. 2015;37(2):122–8.

    Article  PubMed  Google Scholar 

  16. Kao GF, Laskin WB, Olsen TG. Solitary cutaneous plexiform neurilemmoma (schwannoma): a clinicopathologic, immunohistochemical, and ultrastructural study of 11 cases. Mod Pathol. 1989;2(1):20–6.

    CAS  PubMed  Google Scholar 

  17. Koutlas IG, Scheithauer BW. Palisaded encapsulated (“solitary circumscribed”) neuroma of the oral cavity: a review of 55 cases. Head Neck Pathol. 2010;4(1):15–26.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Lisle A, Jokinen C, Argenyi Z. Cutaneous pseudoglandular schwannoma: a case report of an unusual histopathologic variant. Am J Dermatopathol. 2011;33(5):e63–5.

    Article  PubMed  Google Scholar 

  19. Lupton JR, Epps RE, Figueroa P, Kupiec A, Sulica VI. Plexiform neurofibroma with massive hand involvement. J Am Acad Dermatol. 2000;43(6):1136–7.

    Article  CAS  PubMed  Google Scholar 

  20. Luzar B, Falconieri G. Cutaneous malignant peripheral nerve sheath tumor. Surg Pathol Clin. 2017;10(2):337–43.

    Article  PubMed  Google Scholar 

  21. Luzar B, Tanaka M, Schneider J, Calonje E. Cutaneous microcystic/reticular schwannoma: a poorly recognized entity. J Cutan Pathol. 2016;43(2):93–100.

    Article  PubMed  Google Scholar 

  22. 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 

  23. Machado I, Cruz J, Lavernia J, Llombart-Bosch A. Solitary, multiple, benign, atypical, or malignant: the “Granular Cell Tumor” puzzle. Virchows Arch. 2016;468(5):527–38.

    Article  PubMed  Google Scholar 

  24. Miedema JR, Zedek D. Cutaneous meningioma. Arch Pathol Lab Med. 2012;136(2):208–11.

    Article  PubMed  Google Scholar 

  25. Noonan VL, Greene DJ, Brodsky G, Kabani SP. Extraneural sclerosing perineurioma of the buccal mucosa: a case report and clinicopathologic review. Head Neck Pathol. 2010;4(2):169–73.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Pérez-González YC, Pagura L, Llamas-Velasco M, Cortes-Lambea L, Kutzner H, Requena L. Primary cutaneous malignant granular cell tumor: an immunohistochemical study and review of the literature. Am J Dermatopathol. 2015;37(4):334–40.

    Article  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. Rodríguez-Peralto JL, Riveiro-Falkenbach E, Carrillo R. Benign cutaneous neural tumors. Semin Diagn Pathol. 2013;30(1):45–57.

    Article  PubMed  Google Scholar 

  29. Rubin AI, Yassaee M, Johnson W, Elenitsas R, Zaladonis J Jr, Seykora JT. Multiple cutaneous sclerosing perineuriomas: an extensive presentation with involvement of the bilateral upper extremities. J Cutan Pathol. 2009;36(Suppl 1):60–5.

    Article  PubMed  Google Scholar 

  30. Sheth S, Li X, Binder S, Dry SM. Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis. Mod Pathol. 2011;24(3):343–54.

    Article  CAS  PubMed  Google Scholar 

  31. Suchak R, Luzar B, Bacchi CE, Maguire B, Calonje E. Cutaneous neuroblastoma-like schwannoma: a report of two cases, one with a plexiform pattern, and a review of the literature. J Cutan Pathol. 2010;37(9):997–1001.

    Article  PubMed  Google Scholar 

  32. Sundarkrishnan L, Bradish JR, Oliai BR, Hosler GA. Cutaneous cellular pseudoglandular schwannoma: an unusual histopathologic variant. Am J Dermatopathol. 2016;38(4):315–8.

    Article  PubMed  Google Scholar 

  33. Thomas C, Somani N, Owen LG, Malone JC, Billings SD. Cutaneous malignant peripheral nerve sheath tumors. J Cutan Pathol. 2009;36(8):896–900.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pathology, Cleveland Clinic, Cleveland, OH, USA

    Youran Zou & Steven D. Billings

Authors
  1. Youran Zou
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  2. Steven D. Billings
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Correspondence to Steven D. Billings .

Editor information

Editors and Affiliations

  1. Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA

    Steven D. Billings

  2. Departments of Pathology and Dermatology, University of Michigan Medical Center, Ann Arbor, MI, USA

    Rajiv M. Patel

  3. Department of Pathology and Laboratory Medicine, University of Wisconsin–Madison, Madison, WI, USA

    Darya Buehler

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Zou, Y., Billings, S.D. (2019). Nerve Sheath and Related Tumors. In: Billings, S., Patel, R., Buehler, D. (eds) Soft Tissue Tumors of the Skin. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-8812-9_10

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  • DOI: https://doi.org/10.1007/978-1-4939-8812-9_10

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-8810-5

  • Online ISBN: 978-1-4939-8812-9

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