Endometrial stromal sarcoma with sex cord differentiation: An uncommon tumor posing diagnostic challenge

Endometrial stromal sarcoma with sex cord differentiation is a very unusual neoplasm affecting the uterine cavity with an incidence of only 0.25%. They can cause difficulties in diagnosis due to it is histologic appearance and rarity of occurrence. Histopathological diagnosis still remains the cornerstone of diagnosis. Immunohistochemistry and molecular studies are also very helpful. We report a case of a 47-year-old patient who was diagnosed clinically and radiologically as intramural leiomyoma, but on histopathology examination and immunohistochemically proved to be endometrial stromal sarcoma with sex cord differentiation.