Here, we present a case report of a 60-year-old female with a 5-cm tumor in the inferior vena cava (IVC) that was positive for c-kit and CD34 expression. Thus, we considered this to be an extragastrointestinal c-kit-positive stromal tumor (EGIST). To the best of our knowledge, no primary EGISTs of the IVC have been described thus far. The potential occurrence of EGISTs outside the tubular gastrointestinal tract should be recognized in the differential diagnosis of tumors of the great vessels. Thus, we concluded that primary c-kit-positive stromal tumors of the IVC do indeed occur.
Keywords: Extragastrointestinal stromal tumor; Tumor of the inferior vena cava; c-kit-positive tumor.