Current status of primary malignant melanoma of the esophagus: clinical features, pathology, management and prognosis
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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.
KeywordsPrimary malignant melanoma of the esophagus Metastatic malignant melanoma Radical esophagectomy
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.
- 1.Baur EH. Ein Fall von Primaerem Melanoma de Oesophagus. Arb Geb Pathol Anat Inst Tuebingen. 1906;5:343–54.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
- 9.Japan Esophageal Society. Japanese Classification of Esophageal Cancer, tenth edition: part I. Esophagus. 2009;6:1–25.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.PubMedCrossRefGoogle 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
- 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
- 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.PubMedCrossRefGoogle 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.Google Scholar