Abstract Background Primary malignant melanoma of the esophagus (PMME) is a rare disease with a poor prognosis. There are few reports of early-stage cases in which tumor invasion reached the lamina propria or muscularis mucosae, as in the present case. A standard treatment for early-stage PMME has not yet been established. The present study aimed to summarize previous reports and to discuss the indications for surgical treatment of early-stage primary malignant melanoma of the esophagus. Case presentation A 70-year-old woman with PMME was referred to our hospital. She underwent thoracoscopic and laparoscopic subtotal esophagectomy with lymphadenectomy. The resected specimen showed melanocytosis and junctional activity. Melanoma-specific antigens melan-A, S-100, and HMB45 were detected by immunohistochemical staining. The pathological diagnosis was pT1a-MM, pN0, pM0, and pStage IA. She remains alive without evidence of recurrence 39 months later. Conclusion Subtotal esophagectomy with regional radical lymphadenectomy could be recommended to patients with early-stage primary malignant melanoma of the esophagus, and curative surgical resection could improve their prognosis.
