An endoscopic approach to surgical treatment of Ewing’s sarcoma of the sinonasal region in children

Ewing’s sarcoma is a highly aggressive malignant tumor from the group of small-round cell sarcomas, which is most common in children and adolescents. Tumor localization in the maxillofacial region and paranasal sinuses is rare (1-9.8% of cases), which creates difficulties in accumulating clinical experience. Achieving complete resection with negative edges (R0) is a key prognostic factor, however, the anatomical proximity of vital structures limits the possibility of open interventions. A relevant area is the use of minimally invasive video endoscopic transnasal accesses. The presented case demonstrates the effectiveness of a multidisciplinary approach. Achieving R0 resection is a cornerstone of surgical oncology in Ewing’s sarcoma, significantly improving the prognosis. The world literature describes a direct correlation between the radicality of surgery and outcome: incomplete resection (R2) leads to a risk of progression, while radical removal after neoadjuvant therapy allows for long-term remission. The use of endoscopic transnasal access in SJS is a promising but poorly studied area due to the rarity of the pathology. The available data and the presented case indicate that such access allows for adequate local control in complex locations with minimal injury, rapid recovery and a good aesthetic result, which is critically important for compliance with chemotherapy timings in pediatric practice. Ewing’s maxillofacial sarcoma in children is a rare pathology that requires an individualized approach. This clinical example demonstrates that endoscopic transnasal excision is a feasible and effective method of achieving R0 resection after neoadjuvant chemotherapy. The method provides radicality and low injury, however, for final conclusions and standardization of the approach, it is necessary to accumulate further experience and conduct multicenter studies.
Keywords: Ewing’s sarcoma, pediatric oncology, radiation therapy, transnasal endoscopic surgery, Skullbase surgery, otorhinolaryngology, sinonasal region
Conflict of interest. The authors declare no conflict of interest.
Funding. The study was performed without external funding.