Abstract
We report an extremely rare case of malignant peripheral nerve sheath tumor (MPNST) of the esophagus. A 64-year-old woman was found to have enlargement of the ascending aorta on a chest X-ray film taken during an annual checkup. A chest computed tomography (CT) showed a posterior mediastinal tumor, 80×75×40 mm in size and suspected to be a neurogenic tumor based on an endoscopic ultrasound-guided fine-needle aspiration biopsy. Thoracoscopic esophagectomy was performed, and reconstructed by a gastric-tube using the posterior mediastinal route. The tumor was composed of spindle cells with palisaded and steriform patterns. The tumor cells were diffusely positive for S-100 protein, but negative for c-kit, desmin, CD34, and αSMA. We diagnosed low grade esophageal MPNST because the Ki-67 index was 8%. Metastasis was not detected in the regional lymph nodes. The patient has survived for two years with no sign of recurrence. Long term prognosis of esophageal MPNST is unknown, so careful follow-up is needed in the future.
