Abstract Objectives Uterine tumors resembling ovarian sex cord tumors (UTROSCTs) are rare neoplasms. Approximately 60 cases of UTROSCT are reported in the literature. Methods A 53-year-old female was referred to our institution for definitive surgery following an endometrial biopsy diagnosed as adenosarcoma of the uterus. Her history and physical examination were unremarkable. CT scan showed no evidence of metastatic disease. She underwent a staging hysterectomy with bilateral salpingo-oophorectomy. Grossly, a polypoid mass was seen in the uterine cavity with extension into the myometrium. Results Histologic examination of the mass revealed anastomosing cords and nests of epithelioid cells with bland ovoid nuclei, inconspicuous nucleoli, and scant eosinophilic cytoplasm, embedded within a fibromuscular stroma, resembling sex cord stromal tumors of the ovary. Cellular atypia, necrosis, or mitotic activity were not noted. Immunohistochemically, the neoplastic cells expressed cytokeratin AE1/AE3, WT1, CD99, calretinin, inhibin, melan A, and estrogen receptor. These features were consistent with the diagnosis of UTROSCT. Conclusion UTROSCT accounts for less than 0.5% of all uterine malignancies. These tumors arise in perimenopausal females and historically follow a benign course. Although their histomorphologic and polyphenotypic immunoprofile such as sex cord, epithelial, and myoid seems to be unique among uterine mesenchymal neoplasms and can be diagnostically useful, their diagnosis can be confused with more common tumors such as low-grade stromal sarcomas, carcinosarcoma, and adenosarcoma, especially in superficial endometrial biopsies. As in our case, the initial endometrial biopsy, being superficial, revealed endometrial polyp with rare component of sex cord stromal elements, misleading to the diagnosis of adenosarcoma. However, microscopic examination of the hysterectomy specimen revealed the bulk of the sex cord tumor elements within the myometrium, which was not accessible to biopsy. This case, apart from being rare, also highlights the importance of considering this tumor in the differential diagnosis in superficial endometrial biopsies.
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