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Adamantinoma-like Ewing’s sarcoma with EWS-FLI1 fusion gene: a case report

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Abstract

Recent studies have advocated the genotypic and phenotypic delineation of a novel Ewing’s sarcoma histologic variant showing epithelial features defined as “adamantinoma-like Ewing’s sarcoma”. We described an 18-year-old girl with a primary small round-cell sarcoma of the right tibia showing polyphenotypic differentiation with epithelioid features. The neoplastic cells had mainly round or oval nuclei with fine chromatin with a portion of epithelial arrangements. The immunohistochemical analysis showed the epithelial markers of cytokeratin 5/6/18, AE1/AE3, and cytokeratin high molecular weight were stained especially in the foci with epithelioid features, as well as MIC2, S100, and NSE. The diagnosis of the lesion was confirmed as Ewing’s sarcoma by the presence of the EWS-FLI1 fusion transcript, and could be defined as the so-called “adamantinoma-like Ewing’s sarcoma”. After wide excision and high-dose chemotherapy with peripheral blood stem cell transfusion, the patient has been well and continuously event-free for 3 years since the initial diagnosis.

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Abbreviations

VCR:

vincristine

CBDCA:

carboplatin

CYC:

cyclophosphamide

HDCT:

high-dose chemotherapy

IFM:

ifosphamide

I-PAM:

melphalan

NSE:

neuron-specific enolase

PBSCT:

peripheral blood stem cell transfusion

RT-PCR:

reverse transcriptase-polymerase chain reaction

α-SMA:

α-smooth muscle actin

THP:

piarubicin

VP-16:

etoposide

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Acknowledgement

The authors thank Prof. Takayuki Nojima, Department of Pathology, Kanazawa Medical University, for his critical comments on this paper.

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Authors and Affiliations

  1. Department of Orthopedic Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8521, Japan

    Hiromasa Fujii, Kanya Honoki, Akira Kido & Yoshinori Takakura

  2. Department of Diagnostic Pathology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8521, Japan

    Yasunori Enomoto, Takahiko Kasai & Akitaka Nonomura

  3. Hematology and Oncology Unit, Department of Medicine, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8521, Japan

    Itsuto Amano & Makiko Kumamoto

  4. Department of Orthopedic Surgery, National Hospital Organization Nara Medical Center, 840 Shijo-cho, Kashihara, Nara, 634-8521, Japan

    Toru Morishita & Yoshio Mii

Authors
  1. Hiromasa Fujii
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  2. Kanya Honoki
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  3. Yasunori Enomoto
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  4. Takahiko Kasai
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  7. Makiko Kumamoto
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  8. Toru Morishita
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  9. Yoshio Mii
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  10. Akitaka Nonomura
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  11. Yoshinori Takakura
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Correspondence to Kanya Honoki.

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Fujii, H., Honoki, K., Enomoto, Y. et al. Adamantinoma-like Ewing’s sarcoma with EWS-FLI1 fusion gene: a case report. Virchows Arch 449, 579–584 (2006). https://doi.org/10.1007/s00428-006-0279-1

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  • DOI: https://doi.org/10.1007/s00428-006-0279-1

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