Introduction: With an estimated incidence of one to two per one million women, the endometrial stromal sarcoma (ESS) is a rare disease. It is subclassified into a high-grade and a prognostically better low-grade type. Evidence-based data for a standardized therapy is lacking.
Case report: A 32-year-old obese nulligravida presented with persistent vaginal bleeding after the operation of an acute adnextorsion at another hospital. The repeat gynecological ultrasound examination showed a 5.3x5.3x3.6 cm vascularized, partially inhomogeneous mass in the uterus. A fractioned curettage yielded a differential diagnosis of malignant muellerian mixed tumor or a non-differentiated endometrial sarcoma. For completion of the operative treatment, laparotomy with hysterectomy, adnexectomy, and pelvine lymphonodectomy were performed. The final histological report described a 7 cm non-differentiated endometrial sarcoma with infiltration of the left ovary and 25 tumor-free lymph nodes.
Discussion: Standard therapy for resectable sarcoma is abdominal hysterectomy and bilateral adnexectomy. So far, there is little data from studies reporting radio- or chemotherapy treatment of small patient numbers in an adjuvant setting.
Conclusion: The ESS is a very rare disease of the uterus. Due to missing clinical data, it remains a multidisciplinary therapeutic challenge requiring individual decisions. To receive more information on this rare disease, treatment should be performed according to international protocols.