原发性食管恶性黑色素瘤的诊断和治疗

常栋; 胡健; 龚民; 田锋; 王天佑

doi:10.3760/cma.j.issn.1673 9752.2013.10.018

原发性食管恶性黑色素瘤的诊断和治疗

100050 首都医科大学附属北京友谊医院胸外科暨食管疾病中心

基金项目: 首都医科大学基础临床科研合作及北京市李桓英医学基金联合资助项目(10JLL01)

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出版历程

Diagnosis and surgical treatment of primary malignant melanoma in esophagus

Department of Thoracic Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China

摘要

摘要:
目的：总结原发性食管恶性黑色素瘤(PMME)的临床病理特征及其诊断和治疗经验。
方法：回顾性分析2008年1月至2009年6月首都医科大学附属北京友谊医院收治的3例PMME患者的临床资料。采用多种检查诊断，行手术治疗，术后给予辅助性化疗及免疫治疗。
结果：3例患者术前食管钡餐造影检查显示食管下段不规则充盈缺损。食管内镜检查示食管腔内黑紫色包块，直径为3～8 cm。EUS检查示病变起源于黏膜层，呈偏低回声。胸部CT检查显示食管下段呈向心性不均匀增厚，管腔明显狭窄，增强后不均匀强化。患者均行左侧开胸食管部分切除+食管胃吻合术，手术顺利。术后行病理检查确诊，免疫组织化学染色显示瘤细胞抗黑色素瘤特异性单抗(HMB，100蛋白阳性。2例患者生存时间超过3年，1例患者术后6个月因肿瘤广泛转移而死亡。
结论：PMME是一种罕见的食管恶性肿瘤，预后较差，外科手术治疗是首选治疗方法，辅助性化疗和免疫治疗可能延长患者生存时间。
- 食管恶性黑色素瘤 /
- 诊断 /
- 治疗
Abstract:
Objective To investigate the pathological features, diagnosis and treatment of primary malignant melanoma in esophagus (PMME).
Methods The clinical data of 3 patients with PMME who were admitted to the Beijing Friendship Hospital from January 2008 to June 2009 were retrospectively analyzed. All the patients received esophageal barium radiography, electronic fiberesophagoscopy, esophageal endoscopic ultrasonography, computed tomography, and underwent surgery. Adjuvant therapy and immunotherapy were applied postoperatively.
Results The results of barium radiography showed irregular filling defect presentation in the distal esophagus. The results of endoscopy showed a purple black tumor with a length of 3-8 cm in the esophagus. The results of endoscopic ultrasonography showed that the tumor was derived from the mucosal layer with low echo density. The results of computed tomography showed thickness of the lower segment of the esophagus. All the 3 patients received left thoracic approach esophagectomy and esophagogastrostomy. Histopathology and immunohistochemistry confirmed malignant melanoma. The expressions of Mart 1, HMB 45 and S 100 antigen were positive. The survival time of 2 patients was longer than 3 years, and 1 patient died of metastasis 6 months after operation. Conclusions PMME is a kind of rare and malignant tumor with dismal prognosis. Surgical management is the first choice, adjuvant chemotherapy and immunotherapy are hopeful to increase the survival time of the patients.
- Esophageal malignant melanoma /
- Diagnosis /
- Treatment