Gastrointestinal stromal tumors (GIST) are the most common type of gastrointestinal mesenchymal tumor, but are rarely found in the thoracic esophagus. There is no clear consensus about the optimal treatment of this rare disease. A systematic search of the literature was performed for localized esophageal GIST that was resected between 2000 and 2015, and individual patients were included from two major academic institutions. We obtained information on demographics, tumor size and location, mitotic rate, treatment method, and time to recurrence or death. We performed univariate and multivariate Cox regression analyses to evaluate the factors associated with recurrence or death. A total of 28 studies met our inclusion and exclusion criteria, and with two patients from two academic institutions, we had a total of 107 patients in the study. Due to lack of uniformity among studies, there were several missing data for different variables. The average patient age was 56 (n = 98) with mostly males (60%, n = 91). The average tumor size on the CT scan was 7.9 ± 5.4 cm (n = 91), located mostly in the distal esophagus (81%, n = 74). A similar number of patients underwent enucleation (n = 47) compared to esophagectomy (n = 42). Approximately half of the patients had a mitotic rate of 0-4 mitosis per 50 high-powered field (48%, n = 80). The median survival time was 73 months with a 5-year disease free survival of 57% (n = 97). Univariate Cox regression analyses showed that a large tumor, undergoing esophagectomy, and a high mitotic rate were associated with poor survival or recurrence control. We found that patients with a lesion smaller than or equal to 5 cm on the CT scan had a better disease-free survival rate than those with a size greater than 5 cm (HR = 12.41, p = 0.014) and had a 5-year survival rate of 92% with 90% of those patients undergoing enucleation (n = 29). Esophageal GIST is a very rare malignancy. The tumor size and mitotic rate of the tumor are associated with poor survival. However, patients with esophageal GIST measuring 5 cm or smaller may be safely treated with esophageal enucleation.
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