Background: Sarcomas comprise less than 1% of all gynecological cancers and 2% to 5% of all uterine malignancies, of which endometrial stromal sarcoma accounts for approximately 15%. Surgery is fundamental in sarcoma management and a total hysterectomy is performed in the majority of cases.
Case: Presented is a case of low-grade myxoid endometrial stromal sarcoma in a nulliparous adolescent female managed by local resection and uterine reconstruction.
Conclusion: In selected cases, the initial management of a localized and well defined low-grade endometrial stromal sarcoma could potentially be conservative (uterine-sparing) surgical resection.