原发性食管恶性黑色素瘤的外科诊治

Surgical diagnosis and treatment of primary malignant melanoma of the esophagus

目的探讨原发性食管恶性黑色素瘤的临床病理特征、诊断、鉴别诊断和外科治疗。方法回顾我院6例外科手术切除和病理学确诊原发性食管恶性黑色素瘤的相关临床资料,并分析相关文献。结果6例患者中男5例,女1例,年龄48～66岁,平均55.8岁,临床症状主要为吞咽困难和胸骨后疼痛,均经钡餐检查提示食管肿瘤性病变,食管中段4例,下段2例,术前2例误诊为食管癌,4例胃镜刷咬切片检查提示食管恶性黑色素瘤,均行食管切除和胃代食管吻合术。术后病理诊断为原发性食管恶性黑色素瘤,组织学特征与其他部位恶性黑色素瘤相似,免疫组化表达HMB45、S-100、Vimentin,不表达CgA。结论原发性食管恶性黑色素瘤临床罕见,易误诊,诊断依据临床病理特征、影像学检查,内镜刷片检查有利术前诊断,治疗原则为以外科手术切除为主的综合治疗。

Objective To explore the clinical and pathologic characteristics, diagnosis, differential diagnosis, and surgical treatment of primary malignant melanoma of the esophagus, and thereby to improve the level of clinical diagnosis and treatment. Methods The clinical data of relation of 6 cases of primary malignant melanoma of esophagus who had been resected and confirmed by pathologic examination were retrospective analyzed, and the literature was reviewed. Results In 4 patients,5 cases were men, 1 case was woman. Their age ranged from 48 to 66-year-old （ mean 55.8-year-old）. Main clinical symptoms presented dysphagia and retrosternal pain. All of them showed esophageal tumoral lesion of 4 eases middle segment and 2 ease lower segment by means of upper gastrointestinal barium study. 2 patients were misdiagnosed as esophageal cancer preoperatively and 4 patient confirmed esophageal malignant melanoma by endoscopic biopsy. All patients underwent esophgectomy and esophagogastric anastomosis. Postoperatively pathologic examination confirmed the diagnosis of primary malignant melanoma of the esophagus. Immunohistoric inspection displayed HMB45, S-100, and Vimentin, but not CgA. Conclusion Primary malignant melanoma of the esophagus is so rare that it can be easily misdiagnosed. Diagnostic verifying is based on clinical and pathologic feature, and imaging inspection. Endoscopic biopsy contributed to preoperative diagnosis. Therapeutic strategy is the compre- hensive treatment with most likely use of surgical resection dominating.