原发性食管恶性黑色素瘤临床分析

摘要

Objective To investigate clinical characteristics,pathological features,diagnosis and prognosis of malignant melanoma in esophagus (PMME). Methods Clinical data of four PMME patients who received surgical treatment in Beijing Friendship Hospital from May 2008 to April 2018 were analyzed. Clinical manifestations,endoscopic and radiographic examination results,pathological features,treatment methods were recorded,prognosis was observed and relevant literature was reviewed. Results The mean age of the 4 patients (2 males and 2 female) was 57. 8 (47 - 72) years. All patients presented dysphagia. The tumor was single and located in the middle and lower esophagus. The patients underwent esophagectomy and received adjuvant chemotherapy and immunotherapy after operation. Histopathologically,the tumor cells had round or polygonalin shape,with frequent pathological mitosis; melanin granules were visible within the cytoplasm. Immunohistochemical staining showed positive HMB - 45 and Melan - A,partially positive S - 100 and negative CK for tumor cells. The follow - up was 2 to 48months. 3 patients died and 1 lived with disease. Conclusion Primary malignant melanoma of esophagus is a rare and highly malignant tumor with no specific clinical and imaging signs. Immunohistochemical staining helps its diagnosis. Surgery was the crucial part in comprehensive treatment of the disease.%目的 探讨原发性食管恶性黑色素瘤(PMME)的临床特点、病理情况、诊治及预后.方法 回顾性分析2008年5月至2018年4月于首都医科大学附属北京友谊医院收治的4例PMME患者的临床资料,对其临床特点、影像学及内镜表现、病理情况、诊治方法 及预后进行总结并结合文献讨论.结果 4例患者中,男性2例,女性2例;年龄47 ～ 72岁,平均57.8岁;临床表现主要为进食哽噎感.肿瘤均为单发,位于食管中段1例,中下段2例,下段1例;4例患者均接受手术治疗,术后给予辅助性化疗及免疫治疗;肿瘤细胞呈圆形、卵圆形或梭形,核分裂象明显,细胞质内可见黑色素颗粒,邻近食管上皮基底层可见黑色素细胞.免疫表型示瘤细胞弥漫表达HMB - 45、Melan - A,S - 100 散在分布,CK 阴性.随访2 ～ 48 个月,3例死亡,1例无进展生存.结论 PMME是一种罕见的高度恶性肿瘤,临床及影像学表现不特异,确诊有赖于术后病理,以手术为主的综合治疗是主要治疗手段.