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

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