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Current status of primary malignant melanoma of the esophagus: clinical features, pathology, management and prognosis

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Abstract

Primary malignant melanoma of the esophagus (PMME) is a rare disease with an extremely poor prognosis. Up to 2011, approximately 300 cases had been reported worldwide. The average age of onset is 60.5 years old, with a prevalence of males (2:1). A typical finding of PMME is a lobular or polyploid, well-circumscribed and pigmented tumor, partly covered with normal mucosa. PMME represents various colors depending on its melanin quantity and commonly coexists with intramural metastases, melanocytosis or melanoma in situ. The tumor is located from the middle to lower thoracic esophagus. The accuracy of diagnosis from biopsy is approximately 80%, because many cases are misdiagnosed as a poorly differentiated carcinoma because of the absence of melanin granules. A definite diagnosis was made by immunohistochemical examination with positive results of S100 protein, HMB45 and neuron-specific enolase. PMME has a highly metastatic potential, and the incidence of distant metastasis at the initial diagnosis is around 40–80%. A metastatic tumor from cutaneous malignant melanoma is another pigmented esophageal tumor to be considered when making the differential diagnosis for PMME. Junctional activity with melanotic cells in the adjacent epithelium and the presence of in situ melanoma and/or a satellite tumor without a previous history of cutaneous melanoma are definitive. Most of the reported patients were treated with radical esophagectomy, which is believed to be an effective approach for localized PMME. Five-year survival rates have been achieved in 37% recently, while adjuvant therapy has not been proven to increase overall survival but plays a palliative role.

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References

  1. Baur EH. Ein Fall von Primaerem Melanoma de Oesophagus. Arb Geb Pathol Anat Inst Tuebingen. 1906;5:343–54.

    Google Scholar 

  2. Volpin E, Sauvanet A, Couvelard A, Belghiti J. Primary malignant melanoma of the esophagus: a case report and review of the literature. Dis Esophagus. 2002;15:244–9.

    Article  PubMed  CAS  Google Scholar 

  3. Bisceglia M, Perri F, Tucci A, Tardio M, Panniello G, Vita G, et al. Primary malignant melanoma of the esophagus: a clinicopathologic study of a case with comprehensive literature review. Adv Anat Pathol. 2011;18:235–52.

    Article  PubMed  Google Scholar 

  4. Yamazaki K, Ohmori T, Kumagai Y, Makuuchi H, Eyden B. Ultrastructure of oesophageal melanocytosis. Virchows Arch A Pathol Anat Histopathol. 1991;418:515–22.

    Article  PubMed  CAS  Google Scholar 

  5. Ohashi K, Kato Y, Kanno J, Kasuga T. Melanocytes and melanosis of the oesophagus in Japanese subjects—analysis of factors effecting their increase. Virchows Arch A Pathol Anat Histopathol. 1990;417:137–43.

    Article  PubMed  CAS  Google Scholar 

  6. Maekawa T, Satoh K, Maekawa H, Wada R, Matsumoto M. A case of primary malignant melanoma arising in the esophagus. Nippon Gekakei Rengo Gakkai Zasshi. 2005;30:154–9.

    Google Scholar 

  7. Sabanathan S, Eng J, Pradhan GN. Primary malignant melanoma of the esophagus. Am J Gastroenterol. 1989;84:1475–81.

    PubMed  CAS  Google Scholar 

  8. Chalkiadakis G, Wihlm JM, Morand G, Weill-Bousson M, Witz JP. Primary malignant melanoma of the esophagus. Ann Thorac Surg. 1985;39:472–5.

    Article  PubMed  CAS  Google Scholar 

  9. Japan Esophageal Society. Japanese Classification of Esophageal Cancer, tenth edition: part I. Esophagus. 2009;6:1–25.

    Google Scholar 

  10. Taniyama K, Suzuki H, Sakuramachi S, Toyoda T, Matsuda M, Tahara E. Amelanotic malignant melanoma of the esophagus: case report and review of the literature. Jpn J Clin Oncol. 1990;20:286–95.

    PubMed  CAS  Google Scholar 

  11. Joob AW, Haines GK 3rd, Kies MS, Shields TW. Primary malignant melanoma of the esophagus. Ann Thorac Surg. 1995;60:217–22.

    PubMed  CAS  Google Scholar 

  12. Kato N, Kamino K, Yamashita T, Umeyama K, Mitsuhashi T. Primary malignant melanoma of the esophagus. Gan No Rinsho. 1986;32:1459–65.

    PubMed  CAS  Google Scholar 

  13. DiCostanzo DP, Urmacher C. Primary malignant melanoma of the esophagus. Am J Surg Pathol. 1987;11:46–52.

    Article  PubMed  CAS  Google Scholar 

  14. Lohmann CM, Hwu WJ, Iversen K, Jungbluth AA, Busam KJ. Primary malignant melanoma of the oesophagus: a clinical and pathological study with emphasis on the immunophenotype of the tumours for melanocyte differentiation markers and cancer/testis antigens. Melanoma Res. 2003;13:595–601.

    Article  PubMed  CAS  Google Scholar 

  15. Li B, Lei W, Shao K, Zhang C, Chen Z, Shi S, et al. Characteristics and prognosis of primary malignant melanoma of the esophagus. Melanoma Res. 2007;17:239–42.

    Article  PubMed  Google Scholar 

  16. Crippa F, Leutner M, Belli F, Gallino F, Greco M, Pilotti S, et al. Which kinds of lymph node metastases can FDG PET detect? A clinical study in melanoma. J Nucl Med. 2000;41:1491–4.

    PubMed  CAS  Google Scholar 

  17. Horowitz M, Nobrega MM. Primary anal melanoma associated with melanosis of the upper gastrointestinal tract. Endoscopy. 1998;30:662–5.

    Article  PubMed  CAS  Google Scholar 

  18. Walter A, van Rees BP, Heijnen BH, van Lanschot JJ, Offerhaus GJ. Atypical melanocytic proliferation associated with squamous cell carcinoma in situ of the esophagus. Virchows Arch. 2000;437:203–7.

    Article  PubMed  CAS  Google Scholar 

  19. Dumas O, Barthelemy C, Billard F, Dumollard JM, Boucheron S, Calmard P, et al. Isolated melanosis of the esophagus: systematic endoscopic diagnosis. Endoscopy. 1990;22:94–5.

    Article  PubMed  CAS  Google Scholar 

  20. Sharma SS, Venkateswaran S, Chacko A, Mathan M. Melanosis of the esophagus. An endoscopic, histochemical, and ultrastructural study. Gastroenterology. 1991;100:13–6.

    PubMed  CAS  Google Scholar 

  21. Chang F, Deere H. Esophageal melanocytosis morphologic features and review of the literature. Arch Pathol Lab Med. 2006;130:552–7.

    PubMed  Google Scholar 

  22. Mills SE, Cooper PH. Malignant melanoma of the digestive system. Pathol Annu. 1983;18(Pt 2):1–26.

    PubMed  Google Scholar 

  23. Scotto J, Fraumeni JF Jr, Lee JA. Melanomas of the eye and other noncutaneous sites: epidemiologic aspects. J Natl Cancer Inst. 1976;56:489–91.

    PubMed  CAS  Google Scholar 

  24. Dasgupta TK, Brasfield RD. Metastatic melanoma of the gastrointestinal tract. Arch Surg. 1964;88:969–73.

    Article  PubMed  CAS  Google Scholar 

  25. Sanchez AA, Wu TT, Prieto VG, Rashid A, Hamilton SR, Wang H. Comparison of primary and metastatic malignant melanoma of the esophagus: clinicopathologic review of 10 cases. Arch Pathol Lab Med. 2008;132:1623–9.

    PubMed  Google Scholar 

  26. Allen AC, Spitz S. Malignant melanoma; a clinicopathological analysis of the criteria for diagnosis and prognosis. Cancer. 1953;6:1–45.

    Article  PubMed  CAS  Google Scholar 

  27. Lerut T, Nafteux P, Moons J, Coosemans W, Decker G, De Leyn P, et al. Three-field lymphadenectomy for carcinoma of the esophagus and gastroesophageal junction in 174 R0 resections: impact on staging, disease-free survival, and outcome: a plea for adaptation of TNM classification in upper-half esophageal carcinoma. Ann Surg. 2004;240:962–72 (discussion 72–4).

    Google Scholar 

  28. Miyatani H, Yoshida Y, Ushimaru S, Sagihara N, Yamada S. Slow growing flat-type primary malignant melanoma of the esophagus treated with cap-assisted EMR. Dig Endosc. 2009;21:255–7.

    Article  PubMed  Google Scholar 

  29. Suzuki H, Nagayo T. Primary tumors of the esophagus other than squamous cell carcinoma—histologic classification and statistics in the surgical and autopsied materials in Japan. Int Adv Surg Oncol. 1980;3:73–109.

    PubMed  CAS  Google Scholar 

  30. Hamdy FC, Smith JH, Kennedy A, Thorpe JA. Long survival after excision of a primary malignant melanoma of the oesophagus. Thorax. 1991;46:397–8.

    Article  PubMed  CAS  Google Scholar 

  31. Khoury-Helou A, Lozac’h C, Vandenbrouke F, Lozac’h P. Primary malignant melanoma of the esophagus. Ann Chir. 2001;126:557–60.

    Article  PubMed  CAS  Google Scholar 

  32. Uetsuka H, Naomoto Y, Fujiwara T, Shirakawa Y, Noguchi H, Yamatsuji T, et al. Primary malignant melanoma of the esophagus: long-term survival following pre- and postoperative adjuvant hormone/chemotherapy. Dig Dis Sci. 2004;49:1646–51.

    Article  PubMed  Google Scholar 

  33. Suehs OW. Malignant melanoma of the esophagus. Trans Annu Meet Am Bronchoesophagol Assoc. 1961;41:86–93.

    PubMed  CAS  Google Scholar 

  34. De Mik JI, Kooijman CD, Hoekstra JB, Tytgat GN. Primary malignant melanoma of the oesophagus. Histopathology. 1992;20:77–9.

    Article  PubMed  Google Scholar 

  35. Gupta V, Kochhar R, Sinha SK, Das A. Primary malignant melanoma of the esophagus: long-term survival after radical resection. J Thorac Oncol. 2009;4:1180–2.

    Article  PubMed  Google Scholar 

  36. Cheung MC, Perez EA, Molina MA, Jin X, Gutierrez JC, Franceschi D, et al. Defining the role of surgery for primary gastrointestinal tract melanoma. J Gastrointest Surg. 2008;12:731–8.

    Article  PubMed  Google Scholar 

  37. Saba HI, Cruse CW, Wells KE, Klein CJ, Reintgen DS. Treatment of stage IV malignant melanoma with dacarbazine, carmustine, cisplatin, and tamoxifen regimens: a University of South Florida and H. Lee Moffitt Melanoma Center Study. Ann Plast Surg. 1992;28:65–9.

    Article  PubMed  CAS  Google Scholar 

  38. McClay EF, Mastrangelo MJ, Berd D, Bellet RE. Effective combination chemo/hormonal therapy for malignant melanoma: experience with three consecutive trials. Int J Cancer. 1992;50:553–6.

    Article  PubMed  CAS  Google Scholar 

  39. Del Prete SA, Maurer LH, O’Donnell J, Forcier RJ, LeMarbre P. Combination chemotherapy with cisplatin, carmustine, dacarbazine, and tamoxifen in metastatic melanoma. Cancer Treat Rep. 1984;68:1403–5.

    PubMed  CAS  Google Scholar 

  40. Lens MB, Eisen TG. Systemic chemotherapy in the treatment of malignant melanoma. Expert Opin Pharmacother. 2003;4:2205–11.

    Article  PubMed  CAS  Google Scholar 

  41. Kirkwood JM, Ibrahim JG, Sondak VK, Richards J, Flaherty LE, Ernstoff MS, et al. High- and low-dose interferon alfa-2b in high-risk melanoma: first analysis of intergroup trial E1690/S9111/C9190. J Clin Oncol. 2000;18:2444–58.

    PubMed  CAS  Google Scholar 

  42. Kyte JA, Gaudernack G, Dueland S, Trachsel S, Julsrud L, Aamdal S. Telomerase peptide vaccination combined with temozolomide: a clinical trial in stage IV melanoma patients. Clin Cancer Res. 2011;17:4568–80.

    Google Scholar 

  43. Kluger HM, Dudek AZ, McCann C, Ritacco J, Southard N, Jilaveanu LB, et al. A phase 2 trial of dasatinib in advanced melanoma. Cancer. 2011;117:2202–8.

    Article  PubMed  CAS  Google Scholar 

  44. Robert C, Thomas L, Bondarenko I, O’Day S, Weber J, Garbe C, et al. Ipilimumab plus dacarbazine for previously untreated metastatic melanoma. N Engl J Med. 2011;364:2517–26.

    Article  PubMed  CAS  Google Scholar 

  45. Alexandrescu DT, Ichim TE, Riordan NH, Marincola FM, Di Nardo A, Kabigting FD, et al. Immunotherapy for melanoma: current status and perspectives. J Immunother. 2010;33:570–90.

    Article  PubMed  CAS  Google Scholar 

  46. Kottschade LA, Suman VJ, Amatruda T 3rd, McWilliams RR, Mattar BI, Nikcevich DA, et al. A phase II trial of nab-paclitaxel (ABI-007) and carboplatin in patients with unresectable stage IV melanoma: a North Central Cancer Treatment Group Study, N057E(1). Cancer. 2011;117:1704–10.

    Article  PubMed  CAS  Google Scholar 

  47. Kirkwood JM, Gonzalez R, Reintgen D, Clingan PR, McWilliams RR, de Alwis DP, et al. A phase 2 study of tasisulam sodium (LY573636 sodium) as second-line treatment for patients with unresectable or metastatic melanoma. Cancer. 2011.

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Acknowledgments

The authors express sincere thanks to M. Matsukawa and M. Swart for technical assistance and cooperation.

Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Esophageal and Gastroenterological Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan

    Yoshimi Iwanuma, Natsumi Tomita, Takayuki Amano, Fuyumi Isayama & Yoshiaki Kajiyama

  2. Cancer Center, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan

    Masahiko Tsurumaru

  3. Department of Human Pathology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan

    Takuo Hayashi

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  1. Yoshimi Iwanuma
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Correspondence to Yoshimi Iwanuma.

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Iwanuma, Y., Tomita, N., Amano, T. et al. Current status of primary malignant melanoma of the esophagus: clinical features, pathology, management and prognosis. J Gastroenterol 47, 21–28 (2012). https://doi.org/10.1007/s00535-011-0490-y

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  • DOI: https://doi.org/10.1007/s00535-011-0490-y

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