Primary malignant melanoma of the esophagus

Primary malignant melanoma of the esophagus is an uncommon neoplasm. The world literature reports only 139 cases. It is mainly a disease of older age, predominantly in the 6th and 7th decades, with a male-to-female ratio of 2 to 1. Of all published cases, 60 (43.16%) occurred in the lower third, 40 (28.77%) in the middle third, 25 (17.98%) at the junction of the middle and lower thirds, and 14 (10.07%) in the upper third. Dysphagia was the commonest symptom and was found in 79.5% of cases. The tumors were polypoid, pedunculated, often pigmented, and usually presented as clinically advanced neoplasms. The histological and cytological features were similar to those of lentigo maligna melanoma, but with a more aggressive biological behavior. Hematogenous and lymphatic metastases were common. The overall survival was 9.8 months, and two-thirds of the patients died within a year of diagnosis, irrespective of therapeutic modalities. Deaths were disease-related in 85% of cases. It is concluded that, for operable patients, radical surgical resection is the treatment of choice, either for palliation or for cure, with a 5-yr survival of 4.2%. Irradiation is useful as a palliative or adjuvant measure. The roles of chemotherapy and immunostimulation need further evaluation.