Leiomyosarcoma of the esophagus is a rare neoplasm, with only 95 cases having been reported in the literature. Dysphagia is the most commonly noted symptom, however, because of its location in the submucosal layer, the tumor has usually grown to a considerable size by the time this presents. We report herein a case of a 39-year-old man who had no symptoms other than a 7 month history of a cough. After several investigations, the patient underwent resection of the thoracic and abdominal esophagus with lower lobectomy of the right lung through a right and left thoracotomy. The tumor measured 18 x 15 x 8 cm in length and weighed 1,500 g, being the biggest such tumor ever reported. Forty days after the first operation, an extended right hepatic lobectomy of the liver was performed for hepatic metastasis. He was discharged from the hospital 20 days after the second operation and is now doing well. The clinical features and surgical treatment of leiomyosarcoma of the esophagus are discussed herein.

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