Giant High-Grade Endometrial Stromal Sarcoma Myxoid Variant

Introduction: High-grade endometrial stromal sarcoma is characterized by high Cyclin D1 expression and YWHAE-FAM22 genetic fusion. A rare case of myxoid high-grade endometrial stromal sarcoma with fertility-sparing surgery is described, adding to the evidence and better understanding one of the aggressive tumors of the uterine corpus. Case Presentation: A 36-year old nulliparous woman with desire to have children, rapid abdominal growth, abdominal pain, and suspicion of leiomyoma underwent laparotomy, where a left intraligamentary tumor of 30cm, 8,5 kg size was removed and the posterior uterine wall reconstructed. Histological, immunohistochemical and genetic tests confirmed high grade endometrial stromal sarcoma, myxoid variant, with high cyclin D1 expression and YWHAE t (10;17) (q22;p13.3) rearrangements. Despite counselling, the patient refused complete hysterectomy. Conclusion: Pre-surgical diagnosis of endometrial sarcomas remains difficult. The use of immunohisto chemistry and genetic profile testing permit description of new variants of these tumors, allowing for appropriate counselling and management of patients.