A Rare Case of Spontaneous Rupture of Endometrial Stromal Sarcoma Resulting in Acute Hemoperitoneum

Background: The majority of articles in the literature reporting on uterine sarcomas have focused on leiomysosarcomas. This report highlights the rare manifestation of the only reported endometrial stromal sarcoma (ESS) rupture resulting in spontaneous hemoperitoneum in a patient with suspected uterine leiomyomata. Case: A 70-year-old female presented as a referral for management of suspected large uterine leiomyomata. Prior to surgical intervention, the patient experienced spontaneous rupture of an undiagnosed sarcoma. Results: The patient underwent emergent hysterectomy, and, on postoperative day 6, she was discharged to home. Histopathologic examination was remarkable for a ruptured 18.5 x 12.8 x 10.5-cm ESS with vascular invasion. She remained stable postoperatively and is under the care of the gynecology-oncology service. Conclusions: The prognosis of ESS, in comparison with leiomyosarcoma, is better; however, in the setting of tumor rupture, ESS can be equally fatal. When evaluating uterine leiomyomata associated with spontaneous hemoperitoneum, ruptured ESS should also be included in the differential diagnosis.