Background: Since low-grade endometrial stromal sarcoma (LGESS) has the most favourable prognosis in comparison to other sarcomas, fertility-sparing surgery in young women has been reported in rare cases. However, such tumours almost always show positive sex steroid receptors and their evolution in the hormonal milieu of pregnancy remains unpredictable.
Case report: We report the case of a 34-year-old woman treated conservatively for LGESS who conceived rapidly after hysteroscopic resection of the tumour. In the post partum period, pelvic pain motivated a laparoscopic exploration which revealed severe peritoneal recurrence.
Conclusion: This case report highlights the possible dramatic evolution of LGESS after pregnancy and suggests that definitive surgery should not be postponed but performed as soon as the diagnosis of ESS has been made.