Recurrence after fertility-preserving surgery for low-grade endometrial stromal sarcoma

Considering the characteristics of low-grade endometrial stromal sarcoma (ESS), such as relative indolent course, younger age at diagnosis and favorable prognosis, the fertility-preserving management of low-grade ESS has been described by several authors. We report a 34-year-old female with stage IB low-grade ESS who developed recurrence 7 years after neoadjuvant high-dose progestin therapy followed by fertility-preserving surgery and postoperative progestin therapy. The patient stopped progestin therapy and was disease free for 4 years after diagnosis but experienced recurrence with peritoneal dissemination at 7 years. Considering the tendency of late recurrence, long-term surveillance is necessary to ensure timely action. Long-term progestin treatment might be considered after complete remission even beyond 5 years. We performed a literature review and found that many of the cases did not meet solid pathological criteria of low-grade ESS. The diagnostic criteria of low-grade ESS (vs endometrial stromal nodule) should be deliberately documented for future studies of this rare disease.