Evaluating the Role of Surgical Resection and Reconstruction in the Management of Ewing sarcoma of the Chest Wall in the Paediatric Population: A Systematic Review.

BACKGROUND AND

OBJECTIVES

Ewing sarcoma is the most common malignant chest wall tumour in the paediatric population. Following neoadjuvant chemotherapy regimens, the role and extent of surgical resection and reconstruction of the chest wall remain unclear.

METHODS

A systematic review was conducted in accordance with PRISMA guidelines across four major literature databases. Data regarding overall survival, rate of recurrence, role of surgery and adjuvant therapy role was extracted.

RESULTS

Three thousand ninety-one studies were initially identified via the search, with 25 single-centre observational studies (458 patients) and 10 retrospective reviews of multicentre trials (961 patients) included in the final analysis. Five-year overall survival ranged from 35% to 90%.

DISCUSSION: Along with chemotherapy, local control with surgical resection provides the cornerstone of the management of Ewing sarcoma of the chest wall in children. The literature favours delaying surgery until after neoadjuvant chemotherapy, and there appear to be limited benefits to extensive resection according to pre-chemotherapy margins, advocated for in current guidelines. Radiotherapy is no longer advocated for all patients, but rather should be guided by surgical margins and the histological response to chemotherapy in the resected specimens. Surgical reconstructive techniques need to be tailored for the growth potential of these paediatric patients to avoid long-term sequalae, including scoliosis and pulmonary restrictive disease.