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Malignant gastrointestinal neuroectodermal tumor in head and neck: two challenging cases with diverse morphology and different considerations for differential diagnosis

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Abstract

Malignant gastrointestinal neuroectodermal tumor (MGNET) is a sarcoma typically involving the gastrointestinal tract with neuroectodermal differentiation and EWSR1-ATF1/CREB1 fusions. Recently, rare MGNET cases were reported in extragastrointestinal sites. We identified 2 cases of MGNET arising in unprecedented laryngeal and intracranial locations, respectively. Both cases showed spindle and epithelioid tumor cells with amphophilic to clear cytoplasm and occasionally prominent nucleoli, arranged in solid, fascicular, and pseudoalveolar patterns. Case 1 exhibited moderate to marked nuclear atypia and focal intraepithelial component. In contrast, case 2 comprised predominantly low-grade epithelioid cells with extensive pseudopapillary structures. Both tumors showed an S100/SOX10-positive and HMB45/melan-A-negative immunoprofile as well as EWSR1-ATF1 fusion. A chief obstacle in diagnosing case 1 was the histologic and immunophenotypic resemblance to melanoma. The striking pseudopapillary architecture and the intracranial location of case 2 rendered differential diagnoses including meningioma and ependymoma. With the peculiar locations and morphology, these cases posed great diagnostic challenge.

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Acknowledgements

We thank Mr. Yung-Chuan Chung (NTUH) and Ms. Shih-Chen Yu (KCGMH) for the technical support.

Funding

This work was in part supported by the research fund from the National Taiwan University Hospital (NTUH 108-S4295) to Dr. Jen-Chieh Lee.

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Authors and Affiliations

  1. Department and Graduate Institute of Pathology, National Taiwan University Hospital, National Taiwan University College of Medicine, 7 Chung-Shan South Road, Taipei, 10002, Taiwan

    Chien-Tzu Kuo & Jen-Chieh Lee

  2. Department of Pathology, Shuang Ho Hospital, Taipei Medical University, Taipei, Taiwan

    Yu-Chien Kao

  3. Department of Pathology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan

    Yu-Chien Kao

  4. Department of Pathology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan

    Hsuan-Ying Huang

  5. Department of Anatomical Pathology, Cheng-Hsin General Hospital, Taipei, Taiwan

    Cheng-Hsiang Hsiao

Authors
  1. Chien-Tzu Kuo
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  2. Yu-Chien Kao
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  3. Hsuan-Ying Huang
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  4. Cheng-Hsiang Hsiao
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  5. Jen-Chieh Lee
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Contributions

Chien-Tzu Kuo and Jen-Chieh Lee designed the research and wrote the paper. Jen-Chieh Lee, Yu-Chien Kao, and Hsuan-Ying Huang performed molecular analysis. Chien-Tzu Kuo, Jen-Chieh Lee, Yu-Chien Kao, Hsuan-Ying Huang, and Cheng-Hsiang Hsiao interpreted the immunohistochemical stains and contributed to the clinical information, tissue materials, radiologic interpretation, and pathologic diagnosis.

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Correspondence to Jen-Chieh Lee.

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Kuo, CT., Kao, YC., Huang, HY. et al. Malignant gastrointestinal neuroectodermal tumor in head and neck: two challenging cases with diverse morphology and different considerations for differential diagnosis. Virchows Arch 481, 131–136 (2022). https://doi.org/10.1007/s00428-022-03274-y

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  • DOI: https://doi.org/10.1007/s00428-022-03274-y

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