Primary High-Grade Endometrial Stromal Sarcoma of the Ovary: A Rare Entity

Abstract

Aim: To present a rare case of primary high-grade endometrial stromal sarcoma (ESS) of the ovary. Due to its rarity, clinical presentation often mimics more common ovarian neoplasms, leading to diagnostic and therapeutic challenges. Case Presentation: We report a case of a 52-year-old nulliparous woman who presented with progressive abdominal distension, weight loss, and a large abdominal mass. Imaging suggested an ovarian carcinoma or sarcoma. The patient underwent extensive surgical debulking, revealing high-grade ESS upon histopathological and immunohistochemical analysis. Despite adjuvant chemotherapy and radiotherapy, she experienced rapid disease progression, developing metastases within six months and ultimately succumbing to the disease. Discussion: Primary ovarian high-grade ESS is poorly understood, with no established treatment guidelines. Management strategies are typically extrapolated from uterine ESS and other ovarian sarcomas. A multimodal approach, including cytoreductive surgery and adjuvant chemotherapy and radiotherapy, may be beneficial. However, high-grade ovarian ESS remains highly aggressive with a high recurrence rate. Conclusion: This case highlights the aggressive nature of primary ovarian high-grade ESS, emphasizing the need for early recognition, multidisciplinary management, and further research to establish optimal treatment strategies.