Extraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature review
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 r...
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Elsevier
2024-12-01
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| Series: | Gynecologic Oncology Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2352578924002169 |
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| author | Wangpan Shi Somaye Zare Cheryl Saenz Omonigho Aisagbonhi |
| author_facet | Wangpan Shi Somaye Zare Cheryl Saenz Omonigho Aisagbonhi |
| author_sort | Wangpan Shi |
| collection | DOAJ |
| description | 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: We present the case of a 54-year-old female with a large pelvic mass connected to the uterine cornua and retroperitoneal soft tissue. Upon surgical debulking, the mass was characterized by infiltrative tumor cells with high nuclei-to-cytoplasmic ratio invading from the serosal surface into the myometrium. The initial diagnostic work-up was muddied by radiographic suggestion of uterine origin, histologic identification of uterine involvement and diffuse positivity for Cyclin D1, KIT and CD99, which raised the diagnostic possibility of high-grade endometrial stromal sarcoma. However, molecular testing that revealed EWSR1::FLI 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. |
| format | Article |
| id | doaj-art-89c221fd93c840b0ae774ecbaee7f993 |
| institution | DOAJ |
| issn | 2352-5789 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Gynecologic Oncology Reports |
| spelling | doaj-art-89c221fd93c840b0ae774ecbaee7f9932025-08-20T02:39:31ZengElsevierGynecologic Oncology Reports2352-57892024-12-015610153710.1016/j.gore.2024.101537Extraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature reviewWangpan Shi0Somaye Zare1Cheryl Saenz2Omonigho Aisagbonhi3Department of Pathology, University of California San Diego, La Jolla, CA, USA; Corresponding author.Department of Pathology, University of California San Diego, La Jolla, CA, USAMoores Cancer Center, University of California San Diego, La Jolla, CA, USA; Department of Obstetrics, Gynecology and Reproductive Sciences, University of California San Diego, La Jolla, CA, USADepartment of Pathology, University of California San Diego, La Jolla, CA, USA; Moores Cancer Center, University of California San Diego, La Jolla, CA, USABackground: 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: We present the case of a 54-year-old female with a large pelvic mass connected to the uterine cornua and retroperitoneal soft tissue. Upon surgical debulking, the mass was characterized by infiltrative tumor cells with high nuclei-to-cytoplasmic ratio invading from the serosal surface into the myometrium. The initial diagnostic work-up was muddied by radiographic suggestion of uterine origin, histologic identification of uterine involvement and diffuse positivity for Cyclin D1, KIT and CD99, which raised the diagnostic possibility of high-grade endometrial stromal sarcoma. However, molecular testing that revealed EWSR1::FLI 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.http://www.sciencedirect.com/science/article/pii/S2352578924002169EwingUterusEndometrialStromalSarcomaCancer |
| spellingShingle | Wangpan Shi Somaye Zare Cheryl Saenz Omonigho Aisagbonhi Extraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature review Gynecologic Oncology Reports Ewing Uterus Endometrial Stromal Sarcoma Cancer |
| title | Extraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature review |
| title_full | Extraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature review |
| title_fullStr | Extraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature review |
| title_full_unstemmed | Extraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature review |
| title_short | Extraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature review |
| title_sort | extraskeletal ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma a case report and brief literature review |
| topic | Ewing Uterus Endometrial Stromal Sarcoma Cancer |
| url | http://www.sciencedirect.com/science/article/pii/S2352578924002169 |
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