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S100-negative epithelioid malignant peripheral nerve sheath tumor with possible perineurial differentiation

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Abstract

Epithelioid malignant peripheral nerve sheath tumor (MPNST) is a rare subtype of MPNST composed of epithelioid cells with abundant cytoplasm. Currently, strong and diffuse immunostaining for S100 protein and SOX10 is generally regarded as a characteristic feature of epithelioid MPNST. However, malignant tumors with epithelioid morphology that arise from a peripheral nerve or a pre-existing benign nerve sheath tumor should be regarded as epithelioid MPNSTs when they do not show characteristic features that definitively lead to other specific diagnoses. Here, we describe 3 cases of epithelioid MPNST in the peripheral nerve or schwannoma that was negative for S100 protein and SOX10 expression. Instead, these tumors were positive for EMA, GLUT1, claudin 1, and cytokeratin to varying degrees, while all of them retained SMARCB1 and H3K27me3 by immunohistochemistry. EMA, GLUT1, and claudin 1 are known markers of perineurial cell differentiation; thus, they could possibly represent epithelioid MPNST with perineurial cell differentiation.

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Funding

This study was supported by JSPS KAKENHI Grant Numbers JP 16K19079 and 19K16570 (KY).

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Authors and Affiliations

  1. Department of Pathology, the Cancer Institute Hospital, Japanese Foundation for Cancer Research, 3-8-31, Ariake, Koto, Tokyo, 135-8550, Japan

    Kyoko Yamashita, Hiroshi Kawachi & Kengo Takeuchi

  2. Division of Pathology, the Cancer Institute, Japanese Foundation for Cancer Research, Tokyo, Japan

    Kyoko Yamashita, Nobuyuki Hiruta, Rikuo Machinami, Hiroshi Kawachi & Kengo Takeuchi

  3. Department of Orthopedic Oncology, the Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan

    Yuki Funauchi, Keiko Hayakawa, Keisuke Ae & Seiichi Matsumoto

  4. Department of Orthopedic Surgery, Tokyo Medical and Dental University, Tokyo, Japan

    Yuki Funauchi

  5. Department of Orthopedic Surgery, Nagoya University Graduate School and School of Medicine, Nagoya, Japan

    Kunihiro Ikuta & Yoshihiro Nishida

  6. Department of Rehabilitation, Nagoya University Hospital, Nagoya, Japan

    Yoshihiro Nishida

  7. Diagnostic Imaging Center, Cancer Institute Hospital of Japanese Foundation for Cancer Research, Tokyo, Japan

    Teruko Ueno

  8. Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan

    Yoshie Shimoyama

  9. Department of Pathology, Toho University Sakura Hospital, Sakura, Japan

    Nobuyuki Hiruta

  10. Department of Pathology, Kawakita General Hospital, Tokyo, Japan

    Rikuo Machinami

Authors
  1. Kyoko Yamashita
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  2. Yuki Funauchi
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  3. Keiko Hayakawa
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  8. Teruko Ueno
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  9. Yoshie Shimoyama
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  10. Nobuyuki Hiruta
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  11. Rikuo Machinami
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  12. Hiroshi Kawachi
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  13. Kengo Takeuchi
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Contributions

K.Y. designed the study and took the lead in writing the manuscript. Y.F, K.H, K.A, S.M, K.I. and Y.N. contributed to sample preparation. T.U. confirmed image findings and designed the figure. Y.S., N.H., R.M. H.K. and K.T confirmed the pathological diagnosis.

Corresponding author

Correspondence to Kyoko Yamashita.

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The authors declare no competing interests.

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Yamashita, K., Funauchi, Y., Hayakawa, K. et al. S100-negative epithelioid malignant peripheral nerve sheath tumor with possible perineurial differentiation. Virchows Arch 480, 1269–1275 (2022). https://doi.org/10.1007/s00428-021-03218-y

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  • DOI: https://doi.org/10.1007/s00428-021-03218-y

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