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Clear Cell Odontogenic Carcinoma Harboring the EWSR1–ATF1 Fusion Gene: Report of a Rare Case

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Abstract

Clear cell odontogenic carcinoma (CCOC) is a rare and aggressive malignant epithelial neoplasm, which occurs most frequently in the mandible of elderly patients. Morphologically, CCOC shares similar characteristics with other clear cell tumors, especially hyalinizing clear cell carcinoma of the salivary glands (HCCC). Both CCOC and HCCC are known to harbor EWSR1 rearrangements, especially the EWSR1–ATF1 gene fusion, which indicates a possible link between the two lesions. So far, this fusion has been demonstrated in five cases of CCOC in the literature. Herein, we add another CCOC case to the literature, which arose in the mandible of an 82-year-old female patient and was proven to harbor the EWSR1–ATF1 gene fusion. Immunohistochemically, this case was focally positive for CK7, CK14, CK19 and p63. The patient was referred to surgical treatment; however, she died of disease 2 months after the diagnosis, thereby demonstrating the aggressive nature of this tumor.

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Acknowledgements

This study was supported by Coordenação de Aperfeiçoamento de Pessoal de Nível Superior–Brasil (CAPES)–Finance Code 001. T. Santana received a scholarship from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) during the period in which this study was carried out.

Funding

This study was supported by Coordenação de Aperfeiçoamento de Pessoal de Nível Superior–Brasil (CAPES)–Finance Code 001. During the preparation of this manuscript, T. Santana received a scholarship from from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq).

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Authors and Affiliations

  1. Department of Oral and Maxillofacial Pathology, School of Dentistry, University of São Paulo–USP, Av. Prof. Lineu Prestes, 2227, São Paulo, SP, 05508-000, Brazil

    Thalita Santana & Marília Trierveiler

  2. Department of Oral and Maxillofacial Surgery, Mandaqui Hospital Complex, São Paulo, SP, Brazil

    Felipe Ledo de Andrade & Maria Carolina de Sousa Melo

  3. Dentistry Division, Clinics Hospital of the Medical School, University of São Paulo Paulo–USP, São Paulo, SP, Brazil

    Glauber Bareia Liberato da Rocha

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  1. Thalita Santana
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  2. Felipe Ledo de Andrade
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  3. Maria Carolina de Sousa Melo
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  4. Glauber Bareia Liberato da Rocha
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  5. Marília Trierveiler
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Correspondence to Marília Trierveiler.

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Santana, T., de Andrade, F.L., de Sousa Melo, M.C. et al. Clear Cell Odontogenic Carcinoma Harboring the EWSR1–ATF1 Fusion Gene: Report of a Rare Case. Head and Neck Pathol 14, 847–851 (2020). https://doi.org/10.1007/s12105-019-01103-4

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  • DOI: https://doi.org/10.1007/s12105-019-01103-4

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