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

Abstract

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.