primary ewing’s sarcoma of the spine in pediatric patients: a case series analysis and literature review

Abstract

Ewing’s sarcoma is a common malignancy of the bone and soft tissues in pediatric patients. It mostly affects the long bones and pelvis, and less commonly the flat bones and vertebrae. Primary Ewing's sarcoma affecting the spine is very rare. The patient has non-specific symptoms for a prolonged period of time before the correct diagnosis is given. Patients can present with acute paraplegia due to spinal cord compression, which needs prompt surgical intervention. Early diagnosis and treatment is important for neurological recovery. The definitive management includes three main modalities: surgery, radiotherapy, and combination chemotherapy. Adequate surgical excision may not be feasible because of anatomical limitations and local control is mainly achieved by radiotherapy. Because of the low incidence of these tumors, a multitude of therapeutic strategies have been employed with varying success. Currently there are no clinical guidelines outlining optimal management. We present a series of six cases of primary Ewing's sarcoma of the spine, analysis of presenting complaints, examination findings, diagnostic investigations, treatment, outcome and review of relevant literature. Five out of the six patients had prolonged musculoskeletal symptoms before they were correctly diagnosed and three presented with acute paraplegia. All have received aggressive multimodality therapy with complete neurological recovery and are surviving.