We report a case of primary malignant melanoma of the esophagus (PMME). A 67-year-old man was referred to our hospital because of dysphagia. Upper gastrointestinal endoscopy revealed a polyploid tumor in the lower thoracic esophagus. Biopsy specimens revealed large atypical cells containing brown granules, and immunohistochemical staining was positive for HMB-45. Pigmented lesions of the skin, rectum, or eyes were not observed, and we diagnosed the tumor as PMME. A subtotal esophagectomy via right thoracotomy and laparotomy was performed. Histopathological examination showed lymph node metastasis and tumor cell invasion to the submucosal layer (T1b). According to the Japanese classification of Esophageal cancer, the pathological stage was stage Ⅱ T1bN1M0. Adjuvant chemotherapy was not performed because of postoperative complications. Fourteen months after the operation, the tumor relapsed in the lung, liver, and retroperitoneum. Chemotherapy was administered, but the patient died of tumor recurrence 20 months after the operation. The prognosis of PMME is poor, as is that for cases of submucosal invasion. Investigation of the long-term prognosis and efficacy of progressive immunochemotherapy are very important to improve the prognosis of PMME.

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