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OBJECTIVE To investigate the clinical and pathologic features of rectal gastrointestinal stromal tumors (GIST) and to evaluate their reasonable management.METHODS The clinical and pathological data for 19 patients with rectal GIST over the past 19 years were studied retrospectively.RESULTS The diagnosis of the 19 cases was identified by surgery and pathology. All the rectal GISTs were spindle cell type with immunohistohemical analysis showing positive reactivity for CD117 (100%) and CD34 (73.7%). There were 4 cases of high risk, 3 cases of intermediate risk, 5 cases of low risk and 7 cases of very low risk of aggressive behavior in this study.CONCLUSION Rectal GIST, without specific symptoms in the early stage, has a low incidence and usually shows low risk of aggressive behavior. It is difficult to produce an accurate pathological diagnosis before operation and it is difficult to decide whether to save the sphincter before or during operation. Reasonable initial treatment includes trans-anal local resection as the best recommend management of low risk submucosal rectal GIST (＜3.0 cm).