我国原发性食管胃肠间质瘤的研究现状和进展

Current research status and progress of primary esophageal gastrointestinal stromal tumors in China

原发性食管胃肠间质瘤（GIST）是具有不同于其他间叶源性肿瘤的临床病理学和分子生物学特征的少见独立疾病。本文检索和复习相关报道，对我国食管GIST的发病趋势、特征、诊断和治疗的现状和进展进行综述。资料显示，食管GIST的发病率和检出率呈上升趋势；影像学具有一定的特征，超声内镜和CT检查是发现和提示食管GIST的主要诊断方法和有效检查手段；病理和免疫组织化学检测是确诊的证据：风险度分级是选择手术方法和预测预后的重要依据；手术是主要治疗方法，其中〈3cm、极低和低危风险患者可选择内镜切除．3～5cm大多行肿瘤切除，胸腔镜有作为手术首选的趋势；〉5cm、中、高危风险患者宜行食管部分切除术。手术结合靶向治疗和新辅助治疗是主要治疗模式和研究方向。

Primary esophageal gastrointestinal stromal tumors （GIST） is a rare independent disease with clinicopathological and molecular features different from other mesenchymal tumors. Authors searched and reviewed associated reports and summarized the morbidity trends, characteristics, diagnosis and treatment of esophageal GIST in China. Data show that the incidence and detection rate of esophageal GIST presents the rising trend. Imaging has some characteristics. Ultrasonography and CT are main methods and effective examinations to detect and prompt diagnosis of esophageal GIST. Pathology and immunohistochemistry are the evidence for a definite diagnosis. Risk classification is the important basis for selecting surgical methods and predicting prognosis. Surgery is the mainstay treatment. Very low and low risk patients with tumors less than 3 cm can choose endoscopic resection. For tumors of 3 to 5 cm, tumor resection must be considered. Thoracoscopy appears to be the first choice for surgery. Medium and high risk patients with tumors 〉 5 cm should be treated with partial resection of esophagus. Surgery combined with targeted therapy and neoadjuvant therapy is the main treatment pattern and research direction.