Characteristics and prognosis of primary malignant melanoma of the esophagus.

Primary malignant melanoma of the esophagus is an extremely rare but highly aggressive tumor. The preoperative diagnosis is complicated for the lack of specificity. Unfortunately, the prognosis of primary malignant melanoma of the esophagus remains dismal from most literatures. To better understand this special condition, we reviewed the medical records of patients with primary malignant melanoma of the esophagus in our center, retrospectively. Seven cases were seen at Cancer Hospital (Institute) of Chinese Academy of Medical Sciences from 1975 through 2006. Six patients had complete clinical data. Of the six patients, two were females and four were males. The patients mean age was 51 years, ranging from 41 to 60 years. Similar to esophageal carcinoma, dysphagia was the most common symptom. Only one patient, however, was pathologically diagnosed as primary malignant melanoma of the esophagus preoperatively. Surgical procedures were performed to all patients. Among the six patients one accepted radiotherapy perioperatively. Four patients accepted biochemotherapy postoperatively. The most common reason for death was metastasis. Four of the six patients had metastasis to the liver, adrenal gland, heart and lymph nodes, respectively. The survival varied from 5 months to 17 years and the median survival was 8 months. Our data show that primary malignant melanoma of the esophagus is a highly aggressive disease with poor prognosis. Surgery remains the first selected therapy. The role of radiotherapy and chemotherapy in the treatment of primary malignant melanoma of the esophagus is still uncertain.