Multidisciplinary therapy for metastatic primary malignant melanoma of the esophagus: A case report.

Standard treatment strategies have not yet been established for primary malignant melanoma of the esophagus (PMME), and far much less for recurrent disease. There are no reports of anti-programmed death-1 antibody treatment of recurrent PMME. A 60-year-old Japanese man was diagnosed with a primary malignant melanoma in the lower esophagus. The patient underwent mediastinoscope-assisted subtotal esophagectomy, and two nodal involvements were detected in the lymph nodes (LN)s along the left gastric artery. Paclitaxel and oral fluoropyrimidine were administered for 2 months as adjuvant treatment based on results of a histoculture drug response assay. Computed tomography at 8 months after following surgery revealed LN metastasis around the celiac axis. The serum level of the tumor marker 5-S-cysteinyldopa was elevated aberrantly. Although treatment with dacarbazine and interferon-β was initiated, metastatic disease progressed. Therefore, we started anti-programmed death-1 antibody therapy. Following 8 treatment courses, the patient demonstrated a partial response; however, after following 4 more treatment courses, the patient demonstrated progressive disease. Next, hypofractionated radiotherapy was targeted at the metastatic LN and resulted in a partial response. Then, ipilimumab, an anti-cytotoxic T-lymphocyte associated antigen 4, was administered at a dose of 3 mg/kg. After the initial administration of ipilimumab, grade 3 peripheral neuropathy was recognized; thereafter, ipilimumab was not administered. A total of 18 months after following treatment for metastatic LNs, the LN decreased in size, and there were no other signs of metastasis to other organs. The patient then underwent laparoscopic celiac axis lymphadenectomy. Pathological examination of the surgical specimens identified no viable melanoma cells. A total of 8 months after following surgery, he is free from evidence of disease recurrence. This is the first reported case of recurrent PMME successfully treated with multidisciplinary therapy including anti-programmed death-1 antibody therapy, radiotherapy and laparoscopic lymphadenectomy.