Extragastrointestinal stromal tumor in the rectovaginal septum associated with acute arterial occlusion

Abstract Extragastrointestinal stromal tumors (EGISTs) arise from atypical sites, such as the omentum, mesentery, retroperitoneal space, urinary bladder, or rectovaginal septum, and account for fewer than 10% of gastrointestinal stromal tumors (GISTs). Most EGISTs are asymptomatic at the time of diagnosis, due to the fact that they rarely cause symptoms until they grow to greater than 10 cm in diameter. Common presenting symptoms are a feeling of vaginal fullness and increased urinary frequency. Cases described in previous reports have been treated with surgery with or without targeted therapy. Here we report an unusual case of an EGIST at the rectovaginal septum presenting with excessive vaginal bleeding and acute arterial occlusion. This rectovaginal mass was successfully removed using the abdominoperineal approach and did not require targeted therapy.