Objective Endometrial stromal sarcoma (ESS) is an uncommon malignant neoplasm within the female reproductive system. This tumor arises from the stromal cells of the endometrium and ranks as the second most prevalent type of malignant mesenchymal tumor in the uterus, following leiomyosarcoma. It constitutes approximately 1% of all female genital tract cancers and accounts for 3–9% of uterine neoplasms. The clinical specificity of ESS is poor, its imaging manifestations are diverse, and early diagnosis is difficult, relying on pathological confirmation. Methods In this retrospective single-center study, we analyzed the CT and MRI imaging findings of 35 pathologically confirmed ESS cases (26 low-grade, 9 high-grade) to identify characteristic imaging features and subgroup differences. Results Key findings included the uterine rupture/channel sign (observed in 7 of 21 endometrial-involved cases), honeycomb-like vascular shadows (5 cases), and progressive centripetal homogeneous enhancement (2 cases). Low grade ESS tended to present with larger tumors and more frequent extensive pelvic invasion (mean diameter 10.76 ± 6.58 cm vs. 6.57 ± 1.51 cm, P = 0.007), while high grade ESS more commonly presented with abdominal pain (P = 0.013) and elevated CA19-9 (P = 0.013). Conclusion These imaging signs may provide auxiliary clues for preoperative suspicion of ESS; however, definitive diagnosis remains dependent on postoperative pathology and immunohistochemistry. Due to the single-center design, small sample size, and lack of a control group, the diagnostic specificity of these signs requires further validation in multicenter prospective studies.
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