Esophageal melanoma: a systematic review and exploratory recurrence and survival analysis

Esophageal melanoma is a rare and poorly described malignancy. We sought to review all available data on the clinicopathological features, management options, and outcomes of patients with esophageal melanoma to guide clinicians working to treat these uncommon tumors. A systematic literature search of the PubMed, Embase, and Cochrane databases was performed. Exploratory recurrence and survival analyses were performed using previously-validated pooled Cox and logistic regression techniques for case reports and case series. Quality assessment of included studies was performed using the tools developed by the Joanna Briggs and the National Heart, Lung, and Blood Institutes. Fifty-nine studies were reviewed. A total of 93 patients with esophageal melanoma were identified. The mean patient age was 61.2 ± 10.6 years. Esophageal melanoma usually developed at the lower esophagus (48.4%). 90.3% of the patients were symptomatic at presentation, with dysphagia being the most common symptom (72%). Esophagectomy was performed in 91.4% of the patients. Postoperatively, 14 patients (15.1%) received adjuvant chemotherapy. Tumor recurrence was seen in 37 patients (39.8%). The median time to recurrence was 6 months. Disease-specific mortality was 43%. All-cause mortality was 46.1%. On multivariable Cox regression, older patient age (hazard ratio [HR] = 0.91, P = 0.008) and higher Melan-A expression (HR = 0.21; P = 0.029) were associated with a significantly lower risk of mortality. Higher S100 levels (HR = 37.4; P = 0.001) were predictive of poor survival. On logistic regression, large, ulcerated, lower esophageal tumors were significantly more likely to recur (P = 0.018, P = 0.013, and P = 0.027 respectively). Esophageal melanoma is a rare malignancy that tends to present with dysphagia. Most surgically-treated patients undergo esophagectomy. Large, ulcerated, lower esophageal lesions recur more frequently. Immunohistochemistry provides prognostic information regarding survival.