Abstract
Uterine tumors resembling sex-cord tumors are a rare group of tumors with uncertain etiology and histogenesis. The sex-cord tumors are classified into two groups. The first group includes endometrial stromal tumors with foci of sex cord differentiation less than 50% while the second group is composed predominantly or exclusively by sex cord-like elements. We report the case of a middle-aged woman who presented with heavy vaginal bleeding with initial ultrasound findings suggestive of uterine leiomyoma. There was no improvement noticed after a trial of medical treatment; hence, the surgical treatment in the form of total abdominal hysterectomy was undertaken. A few weeks after the surgery, the patient presented with unexplained abdominal pain. Imaging studies demonstrated a hypermetabolic lesion in the upper part of the vagina that was suspicious for malignancy. Complete resection of the mass was performed along with para-aortic lymphadenopathy. Histopathological examination revealed a uterine tumor resembling a sex-cord tumor. Uterine tumors resembling sex-cord tumors are a unique group of uterine neoplasms that exhibits diverge clinical and biological characteristic. Surgical pathologists must recognize this rare entity and differentiate it from other lesions.