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Clinicopathologic characteristics of esophageal primary malignant melanoma

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Abstract

Malignant melanoma is a rare tumor that commonly occurs in the skin; however, it can also develop in other sites, including the esophagus, oral cavity, sinonasal tract, anorectal region, vagina, and eye. Esophageal primary malignant melanoma (EPMM) accounts for approximately 0.2–0.3 % of all malignant esophageal tumors. The mean age of patients who develop EPMM is 61 years (range 21–90 years). EPMM predominantly affects males (male to female ratio 2.4:1). The tumor preferentially occurs in the lower and middle part of the thoracic esophagus and commonly appears as a polypoid mass. Histologically, it is composed of polygonal or spindle cells with atypical nuclei and varied amounts of melanin pigment in the cytoplasm. Immunohistochemical stains for S-100 protein, HMB-45, and Melan-A are useful for a correct pathological diagnosis. EPMM often has scattered lesions and occasionally melanocytosis around the main tumor. EPMM and malignant melanomas occurring in other organs exhibit aggressive biological behavior. A review of autopsy cases indicates that EPMM commonly metastasizes to the lymph nodes (82 %), liver (72 %), lung (69 %), peritoneum (45 %), and bone marrow (41 %). Although improved survival with the molecular targeting agent vemurafenib has been demonstrated in cutaneous melanoma with BRAF V600E mutation, recent evidence indicates that EPMM has genetic alterations unique from those observed in cutaneous and other mucosal melanomas. Since information regarding the genetic features of EPMM is limited, further progress in understanding the molecular pathology of this disease is required for the development of novel therapeutic strategies. This paper aims to review previously reported EPMM cases to clarify its clinicopathologic features that could aid in the development of effective therapeutic strategies.

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Acknowledgments

We thank the Japanese Society of Pathology for providing us with the data from the autopsy cases of esophageal malignant melanoma.

Ethical Statement

About the submission of our manuscript titled “Clinicopathologic characteristics of esophageal primary malignant melanoma”, all of authors here declare that this study was performed in accordance with the World Medical Association and the Declaration of Helsinki.

Conflict of interest

There are no financial or other relations that could lead to a conflict of interest.

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Authors and Affiliations

  1. Department of Pathology, Tokyo Metropolitan Geriatric Hospital, 35-2 Sakaecho, Itabashi-ku, Tokyo, 173-0015, Japan

    Tomio Arai

  2. Department of Pathology, Kyoto Prefectural Medical University, Kyoto, Japan

    Akio Yanagisawa

  3. Department of Pathology, Teikyo University Hospital, Tokyo, Japan

    Fukuo Kondo

  4. Research Team for Geriatric Pathology, Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan

    Junko Aida & Kaiyo Takubo

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  1. Tomio Arai
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  2. Akio Yanagisawa
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  3. Fukuo Kondo
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  4. Junko Aida
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  5. Kaiyo Takubo
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Corresponding author

Correspondence to Tomio Arai.

Additional information

A part of the manuscript was presented at the 62nd annual meeting of the Japan Esophageal Society in June 2008.

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Arai, T., Yanagisawa, A., Kondo, F. et al. Clinicopathologic characteristics of esophageal primary malignant melanoma. Esophagus 13, 17–24 (2016). https://doi.org/10.1007/s10388-015-0499-z

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  • DOI: https://doi.org/10.1007/s10388-015-0499-z

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