• Bowen Li, Hongyu Yang, Xia Hong, Yufan Wang, and Feng Wang.
• Department of Oral and Maxillofacial Surgery, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, China.
• Medicine (Baltimore). 2017 Dec 1; 96 (49): e8988.

RationaleEpithelial-myoepithelial carcinoma (EMC) is regarded as a rare low-grade malignant tumor of the salivary gland, accounting for 0.4% to 1% of all salivary gland tumors. However, epithelial-myoepithelial carcinoma with high-grade transformation (EMC with HGT) is extremely rare, therefore it is easily to be inappropriately diagnosed and treated. Herein, we report an unusual case of EMC with HGT involving the parotid gland and discuss the clinical features and histological characteristic of EMC with HGT, in order to remind the doctors to take appropriate diagnosis and treatment.Patient ConcernsA 77-year-old female sought for treatment in our hospital due to pain mass in the left parotid gland for 6 months with rapid growth recently.DiagnosesEMC with HGT was confirmed by final pathology, and then the result showed there were 2 distinctly different areas in the tumor, including the typical EMC component and intensive spindle cells component.InterventionsThe extensive resection surgery was performed.OutcomesThe patient was uneventful after surgery and no recurrence or metastasis has been observed after follow-up of 4 years.LessonsA review of literature suggested that EMC with HGT patients trend to be more aged, more aggressive and poorer prognosis than typical EMC patients. In order to avoid misdiagnosis and inappropriate treatment, it is necessary to accurately recognize the differences between the EMC with HGT and typical EMC.

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