中国原发性消化道黑色素瘤临床特点汇总分析

Analysis of clinical characteristics of primary gastrointestinal melanoma in China

目的探讨中国原发性消化道黑色素瘤的临床表现、内镜及病理特点和诊断治疗。方法对中国知网(CNKI)检索到的从1983年-2015年449例中国原发性消化道黑色素瘤患者结合大连医科大学附属第二医院收治的2例病例的性别、年龄、部位、内镜特点、病理特点、治疗及转归等进行综合分析。结果原发性消化道黑色素瘤以直肠肛管最为多见,其次为食管。内镜表现:以隆起形或结节样多见,可伴有表面糜烂或污秽;质脆、触之易出血。肿瘤以大于2.0 cm多见,占82.81%,最大径平均大小(4.79±2.67)cm。颜色以褐色和黑色为主,其次为灰白色,部分呈彩色(如蓝色和暗红色等),颜色还可混合存在或呈无色。术前误诊率73.64%。患者1年内死亡率54.19%,其中生存期与大小及浸润深度呈负相关,肿瘤大小与转移、浸润深度呈正相关;生存期与转移及年龄等无关。结论原发性消化道黑色素瘤高度恶性,发现时常为晚期,多伴转移、易被误诊、预后极差,了解其临床表现、内镜、病理及免疫组化等是早期诊断的关键。

Objective To investigate the clinical manifestations, endoscopic manifestations, histological characteristics, diagnosis and treatment of the primary gastrointestinal melanoma in China. Methods The gender, age, position, endoscopic manifestations, histological characteristics, treatment, prognosis of the primary gastrointestinal melanoma were gathered and analyzed by reviewing retrospectively the data of 2 cases seen at our hospital and 449 cases searched in CNKI from 1983 to 2015. Results The primary gastrointestinal melanoma was mostly seen in the rectum and anus, and secondly in the esophagus. A typical endoscopic finding was a protuberant or nodular tumor, with erosion or dirty surface on it. Most lesions were friable and easily hemorrhagic. The maximum diameters of most lesions, accounted for 83.3 %, were larger than 2.0 cm. The average value of the maximum diameters was （4.79 ,严艳 2.67） cm. Most cases presented brown or black, followed by hoar. However, some lesions presented various colors, such as blue, red, even co-exited color or achromatic color. Preoperative misdiagnosed rate was 73.64 % and 1-year mortality was 54.19 %. Negative correlation was observed between survival time and lesion size, infiltration depth. Positive correlation was observed between lesion size and metastasis infiltration depth. Survival time had nothing to do with metastasis and age. Conclusion The primary gastrointestinal melanoma was highly aggressive, and usually found in the terminal age, usually together with extensive metastasis, misdiagnosed and poor prognosis. Knowing the clinical features, endoscopic manifestations, histological and immunohistochemical characteristics is the key to early diagnosis.