Clinicopathological presentations and surgical outcomes of esophageal melanoma

Background: Primary malignant melanoma of the esophagus is a rare tumor with a poor prognosis; the optimal treatment strategy has yet to be established. This study aimed to clarify clinical features, courses, and outcomes of patients undergoing surgical resection of primary malignant melanoma of the esophagus. Methods: Six patients with primary malignant melanoma of the esophagus, in whom the absence of other primary melanomas had been confirmed, were selected from the medical database maintained in Toranomon Hospital. Their clinicopathological characteristics and long-term outcomes were retrospectively reviewed and analyzed. Results: All 6 patients (five males and one female) underwent radical esophagectomy with three-field regional lymphadenectomy, and none received neoadjuvant therapy. Tumor invasion was classified into T1 in 5 (83%) cases and T3 in one (17%). Four (67%) patients had nodal metastases (one N1, one N2 and two N3). No distant metastatic lesions were detected preoperatively in any of our cases. Postoperative surveillance revealed recurrence in all 6 patients, and 5 (83%) died of the disease. The median overall survival was 24 months. One patient with a T3N3M0 tumor was treated after surgery with a dacarbazine-nimustine-vincristine regimen followed by irradiation for bone recurrence and survived for 87 months postoperatively. Another patient with a T1N3M0 tumor who survived for 27 months after liver and bone recurrence was treated with nivolumab, ipilimumab, and dacarbazine. Conclusion: Although the courses of patients with primary malignant melanoma of the esophagus were consistently unfavorable, surgical resection with multidisciplinary therapeutic modalities may prolong survival in some cases.