Primary malignant melanoma of the esophagus is an exceedingly rare disease. This tumor is typically aggressive and disseminates early via the lymphatics and the bloodstream with a mean survival time between 10 and 15 months after radical surgical resection. The role of chemotherapy and immunotherapy is unclear. No treatment plan for the disease has yet been established. Case Report: A 78-year-old man came for a checkup with a medical history of reflux esophagitis and chronic gastritis. Esophagogastroscopy showed a bluishgray tumor of the esophagus, and histology revealed features consistent with malignant melanoma. The patient underwent total transhiatal esophagectomy with curative intention, and esophagogastric anastomosis was performed. Immunohistochemistry revealed tumor cells strongly positive for the melanoma-specific antigen HMB45 and protein S-100, and negative for cytokeratin. A proposed postoperative chemotherapy was declined by the patient. Nine months after surgery, the patient’s condition deteriorated, and a mediastinal lymph node conglomerate was found. Two months later, he died of bleeding into the cervical soft tissue. Conclusion: Up to date, radical surgical resection is the main treatment. Very little is known about the benefits of chemotherapy and immunotherapy. However, these therapeutic modalities may play an important role in the future.

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