Primary malignant bone tumours of spine and pelvis in children

PURPOSE: Axial malignant bone tumours are rare in children and adolescents, and their prognosis is still relatively poor due to non-specific symptoms, such as back or groin pain, which may result in late hospital presentation. Therefore, it is very important to raise awareness regarding this pathology. METHODS: We performed a narrative review, including scientific publications published in English. We searched Medline and Google Scholar databases for information on the incidence and prognosis of axial malignant bone tumours in children and adolescents (< 18 years). Outcomes of different surgical management strategies and reconstruction options were assessed. RESULTS: The incidence of primary malignant bone tumours before the age of 18 years is approximately five per one million population; around 25% of these tumours are located in the axial skeleton. With a five-year survival rate of 50%, tumours in an axial location (chest cage, spine, pelvis) are associated with a poorer prognosis than tumours in more peripheral locations. En bloc excision with clear margins has been shown to improve local control and overall survival, even though obtaining adequate surgical margins is difficult due to the close location of large neurovascular structures and other major organs. Spinal reconstruction options include instrumented fusion with allograft or expandable cage. Pelvic reconstruction is needed in internal hemipelvectomy, and the options include biological, endoprosthetic reconstructions, hip transposition, arthrodesis or creation of pseudoarthrosis and lumbopelvic instrumentation. CONCLUSION: Early diagnosis, a timely adequate multidisciplinary management, appropriate en bloc excision, and reconstruction improve survival and quality of life in these patients. LEVEL OF EVIDENCE: V