Uterine Tumor Resembling Ovarian Sex-Cord Tumor: a Case Report of Recurrence after Conservative Management and Review of the Literature

Background: Uterine tumor resembling ovarian sex-cord tumor is an extremely rare uterine mesenchymal neoplasm. Less than 100 cases have been described in literature and only eleven cases were treated with a fertility sparing approach. Three patients treated with preservation of reproductive function presented a local relapse; among these, two patients were treated conservatively and one had a second relapse with peritoneal carcinomatosis. Case Description: We report the case of a 24-year-old nulliparous woman presenting with abnormal uterine bleeding and secondary anemia. She had no comorbidities or familiar history of gynaecological cancer. Trans-vaginal ultrasound and diagnostic hysteroscopy found a submucosal leiomyoma. Resectoscopic myomectomy was performed but final pathological exam revealed a type II uterine tumor resembling ovarian sex-cord. According to the young age and desire to preserve reproductive function, the patient was submitted to fertility-sparing management and follow up was planned (trans-vaginal ultrasound every three months and CT scan every 6 months). A uterine relapse occurred after 20 months, and the patient underwent laparoscopic hysterectomy with salpingectomy and peritoneal biopsies. After ten months from the radical surgery the patient is alive without disease. This is the twelfth case of a uterine tumor resembling ovarian sex-cord treated with preservation of the uterus and the fourth case of recurrence after a fertility sparing treatment. Conclusions: This report is unique for the completeness of the data provided and for the decision of performing radical surgery after disease recurrence because of the tumor more aggressive behaviour, despite the patient did not achieved a pregnancy yet. The optimal diagnostic path and treatment of this rare disease is still uncertain due to the small number of cases reported in literature but apparently a fertility sparing approach can be offered safely to young patients of reproductive age. In cases of recurrent disease the risk and benefit of an organ preserving surgery should be discussed with the patient based on the data extrapolated from the few cases reported until this moment.