LAUSR

Rationale: A uterine tumor resembling an ovarian sex cord tumor (UTROSCT) is a clinically rare disease with an unclear origin and biological behavior. Patient concerns: We present a case of UTROSCT in a 42-year-old woman who presented with abnormally increased menstrual volume for 2 years. Diagnoses: Initially, only ultrasound examination was performed to diagnose uterine fibroids, and then the tumor was surgically removed and sent for pathological examination. The patient was ultimately diagnosed with UTROSCT mainly based on pathological immunohistochemical examination and was further diagnosed with low malignant potential for recurrence based on genetic testing. Interventions and Outcomes: The patient underwent hysterectomy and bilateral adnexectomy, and no adjuvant radiotherapy or chemotherapy was performed after the surgery. Follow-up to date has indicated that she is in good condition. Lessons: UTROSCT is a rare disease that requires pathological immunohistochemical examination to confirm the diagnosis and genetic testing when necessary so that a clear diagnosis can inform better decision-making regarding treatment measures.