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

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Abstract

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.

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Acknowledgments

The authors would like to thank all the staff in 131 institutions that cooperated in this questionnaire (Table 15). They are also grateful to J. Patrick Barron, Professor Emeritus of Tokyo Medical University, and Adjunct Professor, Seoul National University Bundang Hospital for translating this manuscript.

Ethical Statement

Our studies are performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and subsequent revisions. Comprehensive permission to use the data for the advancement of medicine was obtained from the patients at admission.

Conflict of interest

The authors declare that they have no conflict of interest.

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Correspondence to Hiroyasu Makuuchi.

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Makuuchi, H., Takubo, K., Yanagisawa, A. et al. Esophageal malignant melanoma: analysis of 134 cases collected by the Japan Esophageal Society. Esophagus 12, 158–169 (2015). https://doi.org/10.1007/s10388-015-0484-6

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  • DOI: https://doi.org/10.1007/s10388-015-0484-6

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