SOX-10 and MiTF expression in cellular and ‘mixed’ neurothekeoma

Authors

  • Isabella Fried,

    1. Research Unit Dermatopathology, Department of Dermatology and Venerology, Medical University of Graz, Graz, Austria
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  • Panitta Sitthinamsuwan,

    1. Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand
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  • Sorranart Muangsomboon,

    1. Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand
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  • Steven Kaddu,

    1. Research Unit Dermatopathology, Department of Dermatology and Venerology, Medical University of Graz, Graz, Austria
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  • Lorenzo Cerroni,

    1. Research Unit Dermatopathology, Department of Dermatology and Venerology, Medical University of Graz, Graz, Austria
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  • Timothy H. McCalmont

    Corresponding author
    1. Department of Pathology, University of California, San Francisco, San Francisco, CA, USA
    2. Department of Dermatology, University of California, San Francisco, San Francisco, CA, USA
    • Timothy H. McCalmont, MD,

      Departments of Dermatology and Pathology, University of California, San Francisco, San Francisco, CA 94115, USA

      Tel: +1 415 353 7546

      Fax: +1 415 353 7543

      e-mail: tim.mccalmont@ucsf.edu

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  • (All editorial decisions regarding this manuscript were rendered by Dr. Jennifer McNiff, Associate Editor)

Abstract

Background

Neurothekeoma and nerve sheath myxoma have long been interpreted as related tumors that share nerve sheath linage. Lack of S100 expression in neurothekeoma and similarities of gene expression profiles between neurothekeoma and fibrohistiocytic tumors have created reasonable doubt about this concept. SOX-10 represents a marker for schwannian and melanocytic differentiation, and is expressed in other tumors of nerve sheath linage. Microphthalmia transcription factor (MiTF) expression has been repeatedly reported in cellular neurothekeoma in the recent literature and was proposed as a helpful marker in this entity.

Methods

We investigated 25 cases of cellular neurothekeoma, 8 cases of mixed neurothekeoma and 1 case of nerve sheath myxoma for the expression of SOX-10, MiTF, S100, NKI/C3, Melan-A and smooth muscle actin (SMA) using immunohistochemistry.

Results

A lack of SOX-10 expression was demonstrated in 100% of cellular and mixed neurothekeomas, but was present in the case of nerve sheath myxoma. More than two thirds of neurothekeomas showed very focal or no reactivity with MiTF.

Conclusions

Our data suggest that neurothekeoma and nerve sheath myxoma are unrelated, and that cellular and mixed neurothekeoma may not be of nerve sheath lineage. In addition, MiTF should not be regarded as a useful marker in neurothekeoma.

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