Extraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature review

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Background: Ewing sarcoma is an EWSR1-rearranged aggressive malignancy that occurs commonly in bone and has small round blue cell morphology. A diagnostic challenge is presented in the cases of extraskeletal Ewing sarcoma involving solid organs, such as the uterus. Case report and brief literature review: fusion, indicative of Ewing sarcoma. She was thus referred from the gynecologic oncology service to the soft tissue sarcoma service for adjuvant chemotherapy of vincristine/doxorubicin/cyclophosphamide and ifosfamide/etoposide (VDC/IE). We performed a literature review highlighting immunohistochemical and molecular features along with treatment and outcomes in case series of YWHAE-rearranged high-grade endometrial stromal sarcomas and Ewing sarcoma with uterine and/or retroperitoneal involvement. Conclusion: This case and accompanying literature review highlight the benefit of molecular testing in the context of round cell tumors involving the uterus and the importance of distinguishing high-grade uterine stromal sarcomas from sarcomas of soft tissue origin, as they have different treatment strategies.

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