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Primary malignant esophageal melanoma: Diagnostic and therapeutic manipulations of a Greek patient and review of the literature

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Author(s): P. Cheracakis1, Aikaterini Parasi2, A. Karambelis3, Maria Tzouvala4, Maria Mylonaki1, | F. Georgopoulos1, A. Mastrangelis1, J.K. Triantafillidis1

Journal: Annals of Gastroenterology
ISSN 1108-7471

Volume: 19;
Issue: 1;
Date: 2007;
Original page

ABSTRACT
Primary esophageal melanoma is a rare malignant entity. It has been estimated that since its first description in 1964, no more than 300 cases have been described in the international literature. It represents only 0.1% of all malignant esophageal neoplasms. Amelanotic melanomas represent a minority of all esophageal melanomas. The aim of this presentation is to describe the diagnostic and therapeutic manipulations applied in a patient with amelanotic primary esophageal melanoma. Case report: A man aged 65, was admitted to our department because of dysphagia in solid foods of two months duration. Physical examination revealed nothing important. He was a non-smoker and he denied alcohol consumption. Upper GI barium follow-through showed a feeling defect in the lower third of the esophagus without significant prestenotic dilatation. Upper GI endoscopy revealed the presence of a neoplasm, occupying almost the entire lumen of the esophagus, in an area of at least 5cm. Nevertheless, passing the endoscope through the stenotic lumen of the esophagus could easily be achieved. Histology of tumor samples, revealed the presence of malignant amelanotic esophageal melanoma. Immunohistochemical study showed that the malignant cells were negative in panceratine, ceratine 7 and AE3 (epithelial indices) and positive to Vimentin S100, HMB45 and MART1. Computed tomography of the whole body showed a small metastatic lesion in the liver, without lymph-node involvement. Skin examination revealed nothing important. Ophthalmological examination was negative. Chemotherapy consisting of Cisplatin 90mg/m2 IV (1st day) and Temodal 150mg/m2 tb po (1st and 5th day), every 25 days (3 cycles) combined with radiotherapy were applied. Four months later satellite lesions in the esophagus, and lung and liver metastases were found. Palliation treatment with Interferon- a, was unsuccessful. After one month Fotemustine was given with little improvement. The patient died after 3 more months. Conclusion: Primary amelanotic esophageal melanoma is a rare neoplasm with dismal prognosis. Modern histochemistry can significantly facilitate the correct diagnosis. Key Words: Esophageal melanoma, Esophageal tumors, Malignant melanoma
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