Endometrial stromal sarcoma in a 20-year-old woman

Endometrial stromal sarcoma (ESS) is an uncommon and challenging condition comprising 10% of all uterine sarcomas and found in women 42-58 years of age. ESS is difficult to diagnose in young women as it masquerades as a leiomyoma. We report this tumour in a 20-year-old woman presenting with heavy and prolonged menses and urinary retention. She was not sexually active and did not give consent for pelvic examination. A preoperative diagnosis of a submucous leiomyoma with an adnexal mass was made. At laparotomy, the leiomyoma was found to be wedged between the cervix and the vagina, and was removed vaginally. A 5-6 cm retroperitoneal mass was adherent to the right pelvic wall, which was also removed. Histopathology of both specimens revealed ESS. The final diagnosis according to the International Federation of Gynaecology and Obstetrics classification was stage IV ESS. After oncology consult, she was referred for chemotherapy. She is now on follow-up.