Primary Malignant Melanoma Of Esophagus:One Case Report And Review Of The Literature

Objective:To improve the knowledge of primary malignant melanoma of esophagus in pathogenesis,diagnosis,differential diagnosis and treatment methods.Materials and methods:A case of PMME patient is reported in QiLu hospital of shandong university. Collecting fifty-five PMME cases reported by others,review relevant literatures.Results:In the fifty-five PMME cases, male36cases and female19cases. the patients age from34to79years, the average age is59. The tumors were usually located in the middle and lower third of the esophagus,as the reported cases,11cases of tumors are located in the middle and lower of the esophagus,8cases of tumors are in the middle of the esophagus,36cases of tumors are in the lower of the esophagus. Tumors of the minimal length is2cm, the longest is9cm, the average length of5.08cm. Clinical manifestations of PMME are similar to esophageal cancer. Dysphagia, predominantly for tumors, is the most frequent symptom at presentation. Retrosternal or epigastric discomfort or pain, melena or hematemesis have also been described. Esophagoscopy is the most important auxiliary examination.However, esophagoscopy is difficult to diagnose, the diagnosis rate is very low. Most cases were misdiagnosed as esophageal carcinoma at the initial diagnosis, because of their similar clinical manifestations. According to the diagnostic criteria of primary melanoma proposed by Allen and Spitz, which seems to be widely accepted, the tumor is considered as primary when (a) the mass has a characteristic structure of melanoma containing melanin;(b) the adjacent epithelium contains melanocytes;(c) the tumor is polypoid;(d) it arises from the area of junctional changes in the squamous epithelium. Thus, the proposed diagnosis criteriadepends on the finding of junctional activity adjacent to the tumor mass. Recently, immunohistochemical staining utilizing antibodies specific to HMB-45and/or S-100cytoplasmic protein has been used to identify malignant melanoma of the esophagus. When possible, surgery (curative or palliative), is the preferential method of treatment, further therapy include chemotherapy, chemohormonotherapy, radiotherapy and immunotherapy.50patients are treated with surgery,but only8patients are Followed up.The prognosis is poor; the mean survival after surgery is less than9mo. Most patients die of distant metastasis to the liver, the mediastinum, the lungs and the brain.Conclusion:Primary malignant melanoma of the esophagus is a rare neoplasm and represents0.1%to0.2%of all esophageal malignant tumors. the sexual ratio (male vs. female) is2:1, and an overall mean age is59years old The tumors were usually located in the middle and lower third of the esophagus. The common symptoms were dysphagia, retrosternal pain.Surgical resection is the best treatment of choice.