食管平滑肌肉瘤的诊断与治疗

Diagnosis and treatment of esophageal leiomyosarcomas

目的探讨食管平滑肌肉瘤的诊断和治疗方法。方法总结分析5例食管平滑肌肉瘤的诊断及手术治疗经验,石蜡标本行CD117、CD34、肌特异性肌动蛋白(SMA)免疫组化检查。结果本组仅2例术前经食管镜活检获得病理确诊,2例考虑为食管癌,1例诊断为纵隔肿瘤。术后病理诊断2例合并鳞状上皮癌。3例行食管部分切除获得长期生存;1例行肿物切除加术后放疗,3年半后死亡;1例行探查术,7个月后死亡。免疫组化检查,CD117和CD34均为阴性,4例SMA阴性,1例阳性。结论仅部分食管平滑肌肉瘤术前可以确诊;对于术前未确诊的食管肿瘤和侵犯食管壁而未能确定其来源的纵隔肿瘤,需要术中冰冻病理检查以确定其性质;食管平滑肌肉瘤需行CD117、CD34和SMA等免疫组化检查以排除间质瘤;本病行食管部分切除可获得较满意的疗效。

Objective To explore the methods of diagnosis and treatment of esophageal leiomyosarcoma. Methods The literature was reviewed and the experience of diagnosis and surgical treatment of 5 esophageal leiomyosareoma cases in this hospital was analyzed. The expression of CD117, CD34 and SMA （smooth muscle actin） in paraffin specimen was examined with the immunohistochemical technique. Results Only 2 cases were diagnosed with esophagoscopic biopsy before operation, 2 cases were considered as esophageal carcinoma and 1 case was cansidered as mediastinal tumor. Two cases were associated with squamons cell carcinoma. Three cases received esophageal resection and got long-term survival. One case had the tumor excised and received radiotherapy and died three and a half years later. One case received tumor biospy and died seven months later. In nation, all cases were negative with CD117 and CD34,4 cases were negative with SMA and 1 case was positive with SMA. Conclusions Only a part of esophageal leiomyosarcomas can be diagnosed before operation. Frozen section examination during operation is necessary when esophageal tumors can not be diagnosed before operation or the origin of mediastinal tumor which invades esophageal wall can not be confirmed. The expression of CDll7, CD34 and SMA must be examined with the immunohistochemical technique in esophageal leiomyosarcomas to exclude stromal tumors. Esophageal resection can acquire favourable treatment results.