PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.
PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.