Esophageal malignant melanoma: analysis of 134 cases collected by the Japan Esophageal Society.

Background: Esophageal malignant melanoma is an extremely rare disease and no scientific society has systematically collected data on it previously. Method: The 141 institutes belonging to the Japan Esophageal Society conducted a questionnaire on the details of esophageal malignant melanoma during the 10-year period from 1998 to 2007. Results: The questionnaire return rate was 92.9 %. In the 10-year period, there were 134 cases of the disease, indicating an incidence of about 13-14 cases per year in Japan. Of these, 70 cases (52.2 %) had invasion extending up to the submucosal layer, while 64 cases (47.8 %) were advanced malignant melanoma. There were 84 cases (62.7 %) of lymph node metastasis and, of these, 25 cases (18.7 %) showed metastasis to distant organs. Endoscopic resection was performed in 7 cases (5.2 %) and surgical resection alone and surgery plus chemotherapy were employed in 102 cases (76.1 %), 21 cases (15.7 %) received chemotherapy or chemoradiotherapy and 4 cases received best supportive care. Of these, 53 cases died within 1 year and the 1-, 3-, 5-year survival rates by the Kaplan-Meier method were 56.9, 28.0 and 26.3 %, respectively. Of the 13 cases surviving 5 years or more, 12 were superficial malignant melanoma and 8 had lymph node metastasis. Conclusion: In principle, the treatment of esophageal malignant melanoma consists of surgical resection, but its indications and adjuvant therapy with new drugs require an extremely thorough rethinking in terms of therapeutic strategy. [ABSTRACT FROM AUTHOR]