BACKGROUND Myoepithelial carcinoma (MC) is a rare malignant neoplasm that mainly occurs in the salivary gland. MC can be confused with many other tumors when arising outside the salivary glands because it presents with a wide spectrum of cytomorphological and immunohistochemical features. To the best of our knowledge, esophageal MC has not been previously reported. The purpose of this study was to describe the imaging and clinicopathological features of esophageal MC to improve the understanding of the disease. CASE SUMMARY Three men and one woman diagnosed with esophageal MC were enrolled in this study. The primary clinical symptom was dysphagia. The mass was mainly located in the middle esophagus. Laboratory tests revealed that two patients who underwent tumor abnormal protein were positive. Radical resection was performed for all patients with no adjuvant therapy. Hematoxylin-eosin staining showed infiltrative growth of epithelial cells with hyperchromatic and pleomorphic nuclei toward the periphery. Immunohistochemistry showed that all patients were positive for P63, and most patients were positive for SOX-10, AE1/AE3, P40, and calponin. The Ki-67 values were all higher than 60%. Patient one died one month after discharge from an unknown cause. Patient two lost to follow-up. At patient three’s four-month review, enhanced computed tomography (CT) showed anastomosis recurrence and bilateral lung metastases. He abandoned treatment and lost to follow-up. Patient four attended review appointments regularly and remained in a good general condition. CONCLUSION Here, we present the first report of esophageal MC and review the relevant literature. Esophageal MC is more likely to occur in the middle esophagus in older patients with male dominance. A fungating type observed on CT scanning may help narrow down the differential diagnosis. Cystic change or necrosis may occur in larger lesions. The final diagnosis should be made according to the pathological examination. The treatment for MC is surgical resection, and the efficacy of chemotherapy needs to be determined with future studies.

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