High-Grade Endometrial Stromal Sarcoma in a 16-Year-Old Girl

Abstract Endometrial stromal sarcoma (ESS) is a rare uterine malignancy with a variety of morphologic characteristics and clinical courses. We describe a case of high-grade malignant ESS in an adolescent girl, arising in a rudimentary uterine horn and presenting symptoms of an acute abdomen. The patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy and omentectomy due to a stage IVA high-grade ESS. An adjuvant chemotherapy and radiotherapy treatment was recommended, but 10 days after her discharge the condition of the patient deteriorated, with diffused metastases into the lungs and the abdomen, and finally she succumbed to the disease I month after her first admission to the hospital.