Primary malignant melanoma of the esophagus: a study of clinical features, pathology, management and prognosis.

Primary malignant melanoma of the esophagus (PMME) is a rare disease that is characterized by aggressive invasion, early metastasis, and poor prognosis. Treatment protocols are not well-established. To understand this condition more precisely, we performed a retrospective review of eight cases of PMME diagnosed at the Thoracic Department of the Cancer Center at Sun Yat-Sen University between 1985 and 2009. Eight PMME patients (five men and three women) with a mean age of 58 years (range: 48 to 72 years) were included. Dysphagia was the most common presenting symptom. All patients underwent an Ivor-Lewis esophagogastrectomy and lymph node dissection with postoperative adjuvant chemotherapy. One patient with stage III/pT4N0M0 underwent postoperative chemotherapy plus radiotherapy. Four patients died of distant metastases. The median survival time was 28 months (range: 11 months to 6 years). Our data confirm that PMME is a highly aggressive disease with a poor prognosis. If the diagnosis is suspected or confirmed as PMME, and the patients have no distal metastases or extensive lymph node enlargement, we suggest that surgery should be the first choice of treatment. With regard to adjuvant therapy, we recommend the addition of chemotherapy. The role of radiotherapy remains questionable and requires further investigation.