A Challenging Diagnosis of Endometrial Stromal Sarcoma in a 50-Year-Old Patient: Case Report and Literature Review

Background: Low-grade endometrial stromal sarcoma (LG-ESS) is a rare uterine mesenchymal neoplasm characterized by indolent progression and strong hormonal responsiveness. Accurate diagnosis remains challenging due to its overlapping clinical, pathological, and imaging features with other benign or malignant uterine entities. Case Presentation: This article presents a case of LG-ESS in a 50-year-old woman, encompassing the clinical presentation, imaging features, histopathological and immunohistochemical findings, the surgical management and postoperative course, as well as a focused synthesis of the current literature on this pathology. The patient presented with recurrent abnormal uterine bleeding and secondary anemia. Imaging data, including magnetic resonance imaging (MRI), revealed a heterogeneous intracavitary lesion with cystic components, suggestive of submucosal fibroids. Surgical management via total hysterectomy with bilateral salpingo-oophorectomy was performed due to suspicion of sarcoma and the need to suppress hormonal stimulation. Histopathological and immunohistochemical evaluation confirmed LG-ESS. The patient had no lympho-vascular invasion, presenting a favorable postoperative evolution. Conclusions: This case highlights the need to maintain a strong clinical suspicion for uterine sarcomas in patients presenting with atypical bleeding and presumed fibroids, especially among perimenopausal women. A multidisciplinary approach, including imaging, surgery, pathology, molecular profiling, and oncology, is essential for accurate diagnosis and optimal management.