Abstract
Background: High-grade endometrial stromal sarcoma (HG-ESS) is a homologous uterine sarcoma of a mesenchymal origin. Its natural course is poorly understood, making preoperative diagnosis difficult.
 Case presentation: A 55-year-old nulliparous presented to the gynecological oncology ward as a referral with progressive abdominal distension and pain, constipation, and postprandial vomiting. Computed tomography (CT) scan of the abdomen revealed a bulky uterus with multiple calcified masses with cystic degeneration. A chest CT scan revealed bilateral upper and lower lobe nodules with mediastinum and hilar adenopathy. She was scheduled for total abdominal hysterectomy and bilateral salpingo-oophorectomy. A histopathological examination confirmed HG-ESS. The patient survived for three months following surgery.
 Conclusion: HG-ESS is rare. This case highlights the nonspecific presentation, aggressive nature of the tumor, rapid progression of the disease, and the challenges clinicians face in managing patients who do not honor their appointments with resultant poor overall survival in the late stage.
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