Abstract
BACKGROUND: Low-grade endometrial stromal sarcoma is a rare neoplastic growth in the uterine cavity, representing less than 1% of uterine tumors. Such tumors usually affect premenopausal and perimenopausal women, with a mean age of 46 years. Treatment generally starts with surgical resection of the tumor, followed by chemotherapy, radiotherapy, or hormonal therapy. CASE PRESENTATION: In the current report, we again present a case of low-grade endometrial stromal sarcoma in a 51-year-old Mediterranean woman presenting with abdominopelvic pain. Computed tomography scan revealed a primary uterine tumor measuring 17 × 9 × 9 cm metastasizing to the lungs, bladder, and ureteral orifice, along with lymphovascular involvement. The patient underwent total abdominal hysterectomy, omentectomy, and lymph node dissection. Estrogen deprivation was accomplished by bilateral salpingo-oophorectomy. Lifelong hormonal therapy consisting of letrozole 2.5 mg per day was prescribed, which demonstrated remarkable efficacy, resulting in a partial remission of lung metastasis within 8 months after surgery. Full remission was observed after 18 months of hormonal therapy, with no recurrence. Another scan was performed after 2.5 years, revealing complete remission with no recurrence. CONCLUSION: We again report a case of complete remission of low-grade endometrial stromal sarcoma after surgical removal of the tumor along with first-line hormonal therapy without the use of chemotherapy or radiotherapy, emphasizing the role of hormonal therapy in the treatment of such tumors.