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Dermal Nerve Sheath Myxoma

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  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

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Synonyms

Myxoid neurothekeoma

Definition

Dermal nerve sheath myxoma is a benign nerve sheath tumor with a multinodular growth pattern and myxoid stroma (Harkin and Reed 1969; Fetsch et al. 2005).

Clinical Features

Dermal nerve sheath myxoma typically affects those in their 30s–40s, with an equal predisposition for men and women. With a predilection for the extremities (especially the fingers and the knee), it classically presents as an asymptomatic, painless, slowly growing nodule. Based in the skin or subcutaneous tissues, nerve sheath myxoma is benign, but carries a risk of local recurrences (32%), sometimes multiple (15%), without metastatic disease (Fetsch et al. 2005).

Macroscopy

Most cases are solitary, superficial, multinodular, and range in size from 0.5 to 2.5 cm. Featuring a well-demarcated architecture, dermal nerve sheath myxoma demonstrates a glistening, gelatinous to mucoid cut surface.

Microscopy

Dermal nerve sheath myxoma is a circumscribed tumor consisting of multiple,...

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References and Further Reading

  • Fetsch, J. F., Laskin, W. B., & Miettinen, M. (2005). 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. The American Journal of Surgical Pathology, 29(12), 1615–1624.

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  • Harkin, J. C., & Reed, R. J. (1969). Tumors of the peripheral nervous system. In Atlas of tumor pathology (series 2, fascicle 3). Washington, DC: Armed Forces Institute of Pathology.

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  • Miettinen, M., McCue, P. A., Sarlomo-Rikala, M., Biernat, W., Czapiewski, P., Kopczynski, J., Thompson, L. D., Lasota, J., Wang, Z., & Fetsch, J. F. (2015). Sox10–a marker for not only schwannian and melanocytic neoplasms but also myoepithelial cell tumors of soft tissue: A systematic analysis of 5134 tumors. The American Journal of Surgical Pathology, 39(6), 826–835.

    Article  Google Scholar 

  • Sheth, S., Li, X., Binder, S., & Dry, S. M. (2011). Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis. Modern Pathology, 24(3), 343–354.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

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

    Omar Habeeb & Steven D. Billings

Authors
  1. Omar Habeeb
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  2. Steven D. Billings
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Corresponding author

Correspondence to Steven D. Billings .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Habeeb, O., Billings, S.D. (2020). Dermal Nerve Sheath Myxoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5372-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5372-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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