Here we report a case of primary glomus tumor of the esophagus, which is extremely rare, and review the corresponding literature. A 69-year-old man underwent screening endoscopy during a medical checkup and a submucosal tumor was incidentally found in the midthoracic esophagus. Endoscopic ultrasonography revealed that the tumor originated from the muscle layer of the esophagus. Computed tomography and 18F-fluorodeoxyglucose positron emission tomography revealed neither lymphadenopathy nor distant metastasis. Endoscopic ultrasonography-guided fine needle aspiration biopsy indicated epithelioid mesenchymal tumor. Immunohistochemically, the tumor cells were positive for alpha smooth muscle actin, and focally positive for Discovered on GIST1 but negative for c-kit and protein kinase C theta, suggesting that the lesion was a KIT-negative epithelioid gastrointestinal stromal tumor or glomus tumor. The tumor was successfully enucleated through right thoracotomy. Pathological examination confirmed that the tumor was a glomus tumor of the esophagus. The patient remains healthy without any recurrence to date one year after surgery.
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