Introduction: Most endometrial stromal sarcomas (ESS) develop recurrent disease at pelvis and lung. Recurrent ESS in the cecum causing intussusception is a rare but emergent phenomenon.
Case report: After initial surgical treatment in 1997, a 41-year-old female developed recurrent ESS in the cecum causing ileo-colic intussusception in 2008, which were operated with right hemicolectomy. The patient was then treated with progestin for 2 years to date. She has since been disease-free.
Conclusion: This case report emphasizes the importance of long-term follow-up and initial surgical treatment of ESS and suggests systemic hormonal therapy in case of recurrence.