Schizas Dimitrios, Bagias George, Kanavidis Prodromos, Moris Demetrios, Spartalis Eleftherios, Damaskos Christos, Garmpis Nikolaos, Karavokyros Ioannis, P Misiakos Evangelos, Liakakos Theodore
1st Department of Surgery, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
J BUON. 2020 Jan-Feb;25(1):497-507.
Esophageal gastrointestinal stromal tumors (GISTs) compose a very rare clinical entity, representing 0.7% of all GISTs. Therefore, the clinicopathological factors that affect mortality are currently not adequately examined. We reviewed individual cases of esophageal GISTs found in the literature in order to identify the prognostic factors affecting mortality.
MEDLINE, EMBASE, and the Cochrane Library were systematically searched to identify clinical studies and case reports referring to esophageal GISTs. The clinicopathological features were recorded and evaluated.
A total number of 105 patients were found. The median age of patients was 58 years (mean 52.4%). The majority of patients (71.6%) presented with tumor-associated symptoms. Tumors were mostly located at the lower esophagus (72.9%), and the median tumor size was 7 cm. Esophagectomy was the most common surgical approach (54.3%), followed by tumor enucleation (45.7%). The median follow-up period was 34 months; tumor recurrence occurred in 18 cases (18.9%) and 19 died of disease (19.2%). The overall survival rate was 75.8%. We found out that tumor size and high mitotic rate (>10 mitosis per hpf) were significant prognostic factors for survival. Presence of symptoms, ulceration, and tumor necrosis as well as tumor recurrence were also significant prognostic factors (p<0.01).
Esophageal GISTs' tumor size and mitotic rate are the most significant factors for survival. For dubious cases, a pre-operative biopsy can auspiciously establish the diagnosis of an esophageal GIST. Regarding surgical treatment, tumor enucleation can be safely and feasibly performed for relatively small, intact tumors, whereas large, aggressive tumors are resected with radical esophagectomy.
食管胃肠道间质瘤（GISTs）是一种非常罕见的临床实体，占所有GISTs的0.7%。因此，目前对影响死亡率的临床病理因素研究尚不充分。我们回顾了文献中报道的食管GISTs的个别病例，以确定影响死亡率的预后因素。
系统检索MEDLINE、EMBASE和Cochrane图书馆，以识别涉及食管GISTs的临床研究和病例报告。记录并评估临床病理特征。
共纳入105例患者。患者的中位年龄为58岁（平均52.4%）。大多数患者（71.6%）出现与肿瘤相关的症状。肿瘤大多位于食管下段（72.9%），中位肿瘤大小为7 cm。食管切除术是最常见的手术方式（54.3%），其次是肿瘤摘除术（45.7%）。中位随访期为34个月；18例（18.9%）出现肿瘤复发，19例死于疾病（19.2%）。总生存率为75.8%。我们发现肿瘤大小和高有丝分裂率（每高倍视野>10个有丝分裂象）是生存的重要预后因素。症状的出现、溃疡、肿瘤坏死以及肿瘤复发也是重要的预后因素（p<0.01）。
食管GISTs的肿瘤大小和有丝分裂率是生存的最重要因素。对于可疑病例，术前活检有助于确诊食管GIST。关于手术治疗，对于相对较小、完整的肿瘤，可安全、可行地进行肿瘤摘除术，而对于较大、侵袭性强的肿瘤，则行根治性食管切除术。
