Primary
malignant melanoma of the esophagus (
PMME) is a malignant
tumor which occurs in the
melanin cells of esophageal mucosal epithelial basal layer.
PMME is a
rare disease with an extremely poor prognosis.
PMME represents only 0.1% to 0.2% of all esophageal malignant
tumors.
Dysphagia, retrosternal or epigastric discomfort or
pain is the most frequent symptom at presentation. Retrosternal, epigastric discomfort,
melena or
hematemesis are the major clinical manifestations. The
tumor is often located from the middle to lower thoracic esophagus. The characteristic endoscopic finding of
PMME is a polypoid lesion that is usually pigmented. Immunohistochemical examination with positive results of
S100 protein, HMB45 and
neuron-specific enolase allow a definitive diagnosis.
PMME metastasizes via hematogenic and lymphatic pathways.
Esophagectomy is believed to be an effective approach for localized
PMME. Five-year survival rates of 37% or higher have been achieved recently. Herein, we report a case of an 65-year-old female admitted for progressive difficulty in swallowing for more than 4 mo. After upper gastrointestinal endoscopy and biopsy, upper gastrointestinal series and computed tomography examination, the patient accepted radical
esophagectomy, and the postoperative pathologic and immunohistochemical examination showed
PMME.