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GLI1-altered epithelioid soft tissue tumor: a newly described entity with a predilection for the tongue

  • Author Footnotes
    1 Haoyan Zhong and Chen Xu contributed equally.
    Haoyan Zhong
    Footnotes
    1 Haoyan Zhong and Chen Xu contributed equally.
    Affiliations
    The State Key Laboratory Breeding Base of Basic Science of Stomatology (Hubei-MOST) & Key Laboratory of Oral Biomedicine Ministry of Education, School & Hospital of Stomatology, Wuhan University, Wuhan, Hubei, China

    Department of Oral and Maxillofacial Surgery, School and Hospital of Stomatology, Wuhan University, Wuhan, Hubei, China
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  • Author Footnotes
    1 Haoyan Zhong and Chen Xu contributed equally.
    Chen Xu
    Footnotes
    1 Haoyan Zhong and Chen Xu contributed equally.
    Affiliations
    Department of Stomatology, the First Medical Centre, Chinese PLA General Hospital, Beijing, China
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  • Xinming Chen
    Affiliations
    Department of Pathology, School & Hospital of Stomatology, Wuhan University, Wuhan, Hubei, China
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  • Xiaolong Guo
    Correspondence
    Corresponding author: Xiaolong Guo, DDS, Department of Oral Radiology, School & Hospital of Stomatology, Wuhan University; Tel: 0086-27-87686230; Fax: 0086-27-82195328.
    Affiliations
    The State Key Laboratory Breeding Base of Basic Science of Stomatology (Hubei-MOST) & Key Laboratory of Oral Biomedicine Ministry of Education, School & Hospital of Stomatology, Wuhan University, Wuhan, Hubei, China

    Department of Oral Radiology, School & Hospital of Stomatology, Wuhan University, Wuhan, Hubei, China
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  • Shaodong Yang
    Correspondence
    Shaodong Yang, DDS, Department of Pathology, School & Hospital of Stomatology, Wuhan University, 237 Luoyu Road, Wuhan 430079, Hubei, China Tel: +86278686229; Fax: +862787873260.
    Affiliations
    The State Key Laboratory Breeding Base of Basic Science of Stomatology (Hubei-MOST) & Key Laboratory of Oral Biomedicine Ministry of Education, School & Hospital of Stomatology, Wuhan University, Wuhan, Hubei, China

    Department of Pathology, School & Hospital of Stomatology, Wuhan University, Wuhan, Hubei, China
    Search for articles by this author
  • Author Footnotes
    1 Haoyan Zhong and Chen Xu contributed equally.
Published:October 20, 2021DOI:https://doi.org/10.1016/j.oooo.2021.10.007

      Abstract

      A novel subset of epithelioid soft tissue tumors with GLI1 gene fusions or amplifications has recently been characterized. Histologically, these tumors typically exhibit multinodular or plexiform growth of ovoid, round to epithelioid neoplastic cells, with a distinctive nested architecture separated by a rich delicate arborizing vascular network. The immunoprofile is variable and non-specific, indicating no definitive line of differentiation. GLI1-altered epithelioid soft tissue tumor is considered to be a low grade sarcoma, however, with limited number of cases reported the biologic behaviour of this entity is unclear. Interestingly, these tumors frequently occur in the head and neck, with a clear predilection for the tongue. To date, 11 cases of lingual GLI1-altered epithelioid soft tissue tumors have been reported in the English literature, and none showed evidence of recurrence or metastasis. Herein, we report an additional case of lingual GLI1-altered epithelioid soft tissue tumor harboring GLI1 fusion in a 56-year-old man with regional nodal metastasis and distant metastasis, in order to increase the awareness of this entity among oral pathologists and clinicians. An accumulation of similar cases is mandatory in order to clarify biological behaviors and also for the development of new therapeutic strategies.

      Keywords

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