Diagnostic challenges in uterine tumor resembling ovarian sex cord tumor: Two case reports

Uterine tumors resembling ovarian sex cord tumors (UTROSCTs) are rare neoplasms, with cervical involvement posing exceptional diagnostic challenges. We report two cases: Case 1: a 43-year-old woman presenting with abnormal uterine bleeding and a cervical mass. An initial biopsy misinterpreted the lesion as a perivascular epithelioid cell tumor (PEComa), resulting in modified radical hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymphadenectomy. Subsequent multi-institutional histopathological consultation confirmed cervical UTROSCT. Case 2: a 53-year-old postmenopausal woman underwent hysteroscopic resection for endometrial thickening revealing UTROSCT, prompting total hysterectomy with bilateral salpingo-oophorectomy. Both patients remain disease-free at 72 and 3 months postoperatively. These cases highlight UTROSCT's non-specific imaging features and frequent histomorphologic overlap with other benign and malignant entities, particularly in the cervix, necessitating immunohistochemical differentiation. Case 1 underscores how diagnostic error can lead to overtreatment. Comprehensive immunohistochemical evaluation is critical for accurate diagnosis. Although total hysterectomy remains standard management, fertility-sparing options may be appropriate for select patients. Long-term surveillance is recommended due to the risk of recurrence.