Pleural Ewing’s Sarcoma in Young Women: A Report of Two Cases with Review of the Literature

Abstract

Background: Ewing's sarcoma (ES) arising from the pleura is an exceedingly rare entity, accounting for fewer than 2% of all ES cases. Its rarity and non-specific clinical presentation frequently result in diagnostic delays and therapeutic challenges. Presentation of Cases: We report two young Moroccan female patients with histologically confirmed pleural ES managed at our institution. The first, a 25-year-old woman, presented with a large locally advanced left pleural ES and received six cycles of VIDE (vincristine, ifosfamide, doxorubicin, etoposide) neoadjuvant chemotherapy, achieving an initial response followed by metastatic progression requiring second-line gemcitabine-docetaxel. The second, an 18-year-old woman, presented with a locally advanced right latero-thoracic soft-tissue ES with pleural involvement and was treated with three cycles of VDC-IE (vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide and etoposide), achieving a 73% partial response; surgical resection was declined and she was referred for consolidative radiotherapy. Discussion: Pleural ES is an aggressive malignancy requiring multidisciplinary management. Immunohistochemical confirmation (CD99 positivity) and cytogenetic analysis are essential for diagnosis. Multimodal treatment including chemotherapy, surgery, and radiotherapy remains the cornerstone of management. Conclusion: Our cases highlight the rarity of this tumor, the importance of early diagnosis, and the significant therapeutic challenges posed by locally advanced disease in resource-limited settings.