Extragastrointestinal stromal tumor arising in the rectovaginal septum: report of an unusual case with literature review

Abstract

Background: Extragastrointestinal stromal tumor (EGIST) is a rare tumor that shows features of gastrointestinal stromal tumor (GIST) outside the gastrointestinal tract, but EGIST arising in the rectovaginal septum is extremely rare.

Case: We report a unique case of a 42-year-old woman with EGIST in rectovaginal septum. The tumor was excised locally. The patient is healthy without evidence of recurrence 11 months after surgery.

Conclusion: The clinical features and treatment of EGIST are not well known because few cases have been reported. Pathologically, immunohistochemistry with antibody against c-kit protein (CD117) is valuable for diagnosis of EGIST. Mitotic rate is the most reliable prognostic factor, and mutations in KIT may be a clinically useful adjunct marker in the evaluation. Surgery is the most effective treatment, and long-term follow-up is necessary.