Clear Cell Odontogenic Carcinoma Harboring the EWSR1-ATF1 Fusion Gene: Report of a Rare Case
| Autor: | Maria Carolina de Sousa Melo, Thalita Santana, Felipe Ledo de Andrade, Marília Trierveiler, Glauber Bareia Liberato da Rocha |
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| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty Oncogene Proteins Fusion Odontogenic Tumors Case Reports Pathology and Forensic Medicine Fusion gene 03 medical and health sciences 0302 clinical medicine Fatal Outcome Rare case Medicine Humans Surgical treatment Hyalinizing clear cell carcinoma Clear cell odontogenic carcinoma Aged 80 and over EWSR1/ATF1 Fusion Gene business.industry Malignant Epithelial Neoplasm Carcinoma medicine.disease 030104 developmental biology Oncology Otorhinolaryngology 030220 oncology & carcinogenesis Female business Clear cell |
| Zdroj: | Head Neck Pathol |
| ISSN: | 1936-0568 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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