Amelanotic malignant melanoma of the esophagus. Report of a case.

Primary malignant melanomas of the esophagus are rare neoplasms with aggressive behavior and tendency to lymphatic and hematogenous spread. We report on a patient affected with a primary amelanotic melanoma of the esophagus synchronous to a squamous cell carcinoma of the epiglottis and treated by subtotal esophagectomy. A 58-year-old woman presented with a three-month history of dysphagia, retrosternal pain and weight loss. An upper gastrointestinal endoscopy revealed a 3 cm, non pigmented, polypoid mass of the middle esophagus and biopsy was consistent with undifferentiated carcinoma. Fiberoptic bronchoscopy revealed a 0.5 cm, polypoid lesion of the right aspect of the epiglottis consistent with a squamous cell carcinoma. The esophageal neoplasm was treated by subtotal esophagectomy while the squamous cell carcinoma of the epiglottis was referred to radiotherapy 1 month after surgery. Microscopy showed diffuse proliferation of anaplastic cells with multiple mitotic figures, marked nuclei and multilobulated nucleoli. The neoplasm stained positive for HBM-45 antigen and S-100 protein. Tumor stage was pT1N0M0. Review of patient's medical history and exploration of skin and mucous membranes failed to reveal malignant lesions and definitive diagnosis was primary amelanotic malignant melanoma of the esophagus. The patient died 16 months after surgery of disseminated disease. In conclusion the present case confirms that primary malignant melanomas of the esophagus have a dismal outcome even in cases of early-stage lesions amenable to surgical resection.