Extra Gastrointestinal Stromal Tumor of Uterus: A Rare Presentation.

Gastrointestinal stromal tumors (GISTs) occurring outside the gastrointestinal tract, also termed as extra GISTs (EGISTs) are rare tumors believed to arise from the peritoneum. Here, we present the case of highly aggressive EGISTs mimicking as a leiomyosarcoma of the uterus which is a very rare entity which to our knowledge has been reported once before. The diagnostic dilemma thus created was solved with the help of immunohistochemistry. These tumors are resistant to chemotherapy and radiotherapy but sensitive to targeted therapy with the tyrosine kinase inhibitor, imatinib mesylate and have a better prognosis unlike their closest differential diagnosis namely leiomyosarcoma of the uterus.