Key result
A 55-year-old woman with primary vulvar Ewing sarcoma confirmed by EWSR1 gene rearrangement died nine months after diagnosis despite wide local resection and eight cycles of VDC/IE chemotherapy.
Why the study?
Primary vulvar Ewing sarcoma is exceedingly rare, especially in postmenopausal women, with only 14 cases confirmed through molecular cytogenetic analysis to date.
Case Report (n=1)
This case highlights the importance of considering Ewing sarcoma in the differential diagnosis of vulvar tumors and underscores the value of molecular diagnostics.
Primary vulvar Ewing sarcoma (ES) is exceedingly rare, particularly in postmenopausal women. To date, only 14 cases have been confirmed through molecular cytogenetic analysis. We report the case of a 55-year-old woman presenting with a painful, ulcerated vulvar mass. Imaging revealed a 6×7.9×8.5 cm lobulated soft tissue lesion. Contrast-enhanced computed tomography (CECT) demonstrated pulmonary metastases. Histopathology of the vulvar mass showed sheets of malignant small round cells, which were highlighted by strong membranous CD99 expression and NKX2.2 nuclear expression on immunohistochemistry. Fluorescence in situ hybridization (FISH) using a break-apart probe confirmed EWSR1 gene rearrangement, establishing the diagnosis of ES. Despite receiving eight cycles of chemotherapy, the patient succumbed to the disease. This is the 15th reported case of primary vulvar ES with molecular confirmation. The case highlights the importance of considering ES in the differential diagnosis of vulvar tumors and underscores the value of molecular diagnostics in guiding management.
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Gondi et al. (2025) conducted a case report in Primary vulvar Ewing sarcoma (n=1). Wide local resection and VDC/IE chemotherapy was evaluated on Clinical outcome. A 55-year-old woman with primary vulvar Ewing sarcoma confirmed by EWSR1 gene rearrangement died nine months after diagnosis despite wide local resection and eight cycles of VDC/IE chemotherapy.
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