Primary malignant melanoma of the esophagus is an extremely rare non-epithelial neoplasm with a fatal prognosis. At present, approximately 184 cases have been published in the world literature. Of all published cases, 10 (5.4%) occurred in Spain. A 76 year-old man with malignant melanoma of the esophagus presented with dysphagia and weight loss, and treated by subtotal esophageal resection is reported. There was no history of previous cutaneous melanoma and ophthalmologic examination had been normal. At esophagoscopy a polypoid and pigmented mass (diameter 5 cm) almost completely occluding the lumen in the lower third of the esophagus was found. Multiple tumor biopsies were obtained. Histological diagnoses were made by examining endoscopic biopsy specimens and confirmed with resected specimens. The purpose of this communication is to present a case of primary esophageal malignant melanoma proved preoperatively by histological and immunohistochemical studies (positive reaction to the HMB-45 and S-100 antigen), followed by a review of the literature of these exceptional tumors.
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