Amelanotic malignant melanoma of the esophagus: report of a patient with recurrence successfully treated with chemoendocrine therapy.

We report a case of primary amelanotic malignant melanoma of the esophagus, an extremely rare disease. A 58-year-old man was diagnosed as having middle esophageal cancer with lymph node metastasis, which was classified as esophageal cancer, Stage III:T3N1M0, by International Union Against Cancer (UICC) criteria. Preoperative chemotherapy was performed, but the response assessment was no change (NC). The patient underwent a subtotal esophagectomy via right thoracotomy and laparotomy. Reconstruction was performed by pulling up the stomach via the retrosternal route; the site of anastomosis was the neck. Adjuvant chemotherapy consisted of five courses of dacarbazine (DITC), nimustine (ACNU), vincristine (VCR), and interferon-beta. Eleven months after the surgery, computed tomography (CT) demonstrated recurrence in the upper mediastinum. The patient received chemoendocrine therapy, consisting of the first planned course of DITC, ACNU, and cisplatin (CDDP), given intravenously; and tamoxifen (TAM), given orally. Subsequently with a modified regimen of this therapy he attained a complete response (CR). In general, the prognosis of esophageal malignant melanoma is very poor. Although our patient had a recurrence, he is alive 4 years and 5 months after the surgery and 3 years and 6 months after the recurrence. The chemoendocrine therapy probably contributed to this outcome.