• Xue-Ying Wang, Mao-Chun Zhang, Jiao Chen, and Jiang-Hua Huang.
• Department of Ultrasound, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan ProvinceChina.
• Medicine (Baltimore). 2022 Sep 2; 101 (35): e30414.

RationaleA uterine tumor resembling an ovarian sex cord tumor (UTROSCT) is a clinically rare disease with an unclear origin and biological behavior.Patient ConcernsWe present a case of UTROSCT in a 42-year-old woman who presented with abnormally increased menstrual volume for 2 years.DiagnosesInitially, 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 OutcomesThe 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.LessonsUTROSCT 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.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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