Extracutaneous malignant glomus tumors are unusual, and their occurrence in the esophagus has been recognized with extreme rarity. We describe a 47-year-old man who presented with progressive dysphagia and weight loss. Upper gastrointestinal endoscopy, a barium esophagram, and chest computed tomography revealed an unusually large, aggressive soft mass at the middle portion of the esophagus. A curative resection (R0 resection) was achieved by means of Ivor-Lewis esophagectomy and mediastinal lymph node dissection. The histopathologic examination and immunohistochemical studies demonstrated a malignant glomus tumor of the esophagus with mediastinal lymph node metastases.
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