Introduction: Uterine tumors resembling ovarian sex cord tumors (UTROSCT) are uterine neoplasms of unknown histiogenesis which show near complete differentiation towards ovarian sex cord elements and are postulated to arise from pluripotential uterine mesenchymal cells or endometrial stromal cells with secondary sex cord differentiation.
Case presentation: A 48 year old post-menopausal women presented with abnormal uterine bleeding for which she underwent total abdominal hysterectomy with bilateral salpingo-ophorectomy. Gross examination revealed a gelatinous grayish white tumor confined to the myometrium, 7 cm in maximum dimention. Microscopic examination revealed monomorphic round to oval tumor cells in anastomosing cords and trabeculae with myxoid background. Immunohistochemically, tumor cells showed diffuse positivity for vimentin, CD99 and S100, while focal positivity was seen with pancytokeratin immunostain. The case was diagnosed as UTROSCT. No evidence of metastasis was found on systemic clinical and radiologic workup.
Conclusion: UTROSCT are rare uterine tumors which can be diagnosed with certainty on morphologic and immunohistochemical grounds. It is important to recognize these tumors as they behave differently from endometrial stromal tumors with sex cord like elements (ESTSCLE).
Keywords: CD99; Endometrial stromal tumor with sex cord like elements (ESTSCLE); Uterine tumors resembling ovarian sex cord tumors (UTROSCT).