Upper gastrointestinal endoscopy in a 43-year-old man complaining of a tight sensation on swallowing was found to have a protruding lesion of the lower esophagus, and biopsy indicated granular cell tumor. CT scan showed multiple liver metastases. The tumor was resected by esophagectomy via a left thora-coabdominal approach with jejunal interposition, and liver biopsy was performed. Pathological findings of liver biopsy indicated liver metastases of the granular cell tumor. The diagnosis was malignant granular cell tumor. Chemotherapy administered for the liver metastases was not effective. The patient died 14 months after the operation. Most granular cell tumors are benign tumor. We reported a rare case of malignant granular cell tumor of the esophagus.
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