Abstract
BACKGROUND: Ewing sarcoma of the rib is a rare and aggressive malignancy in children, requiring a multidisciplinary approach for optimal management. Complete tumor resection is crucial for survival but often results in extensive chest wall defects, necessitating complex reconstruction. This case highlights the successful en-bloc resection and simultaneous chest wall reconstruction in a pediatric patient, emphasizing the role of a coordinated surgical strategy. CASE PRESENTATION: A 10-year-old girl presented with progressive chest pain and a palpable anterior thoracic mass. Imaging revealed a large Ewing sarcoma of the left 6th rib with intra- and extrathoracic extension. Following neoadjuvant chemotherapy per the EuroEwing 2012 protocol, she underwent en-bloc resection of the 5th, 6th, and 7th ribs with a wedge lung resection. Chest wall reconstruction was performed using a polypropylene mesh reinforced with a latissimus dorsi flap and an anterior serratus muscle layer. The postoperative course was uneventful, and she remained tumor-free at a two-year follow-up. CLINICAL DISCUSSION: Managing pediatric Ewing sarcoma of the chest wall is challenging due to the need for oncologic clearance while maintaining thoracic stability and function. The choice of reconstructive technique must balance skeletal integrity, pulmonary mechanics, and future growth. In this case, the combination of synthetic mesh and autologous muscle flaps provided structural support and soft tissue coverage, enabling effective adjuvant radiotherapy. A multidisciplinary team was essential for achieving optimal oncological and functional outcomes. CONCLUSION: En-bloc tumor resection with immediate, well-planned reconstruction can lead to favorable oncological and functional outcomes, improving survival and quality of life.