Outcomes in different age groups with primary Ewing sarcoma of the spine: a systematic review of the literature

OBJECTIVE Ewing sarcoma (ES) is among the most prevalent of bone sarcomas in young people. Less often, it presents as a primary lesion of the spine (5%-15% of patients with ES). METHODS A systematic literature search was performed, querying several scientific databases per PRISMA guidelines. Inclusion criteria specified all studies of patients with surgically treated ES located in the spine. Patient age was categorized into three groups: 0-13 years (age group 1), 14-20 years (age group 2), and > 21 (age group 3). RESULTS Eighteen studies were included, yielding 28 patients with ES of the spine. Sixty-seven percent of patients experienced a favorable outcome, with laminectomies representing the most common (46%) of surgical interventions. One-, 2-, and 5-year survival rates were 82% (n = 23), 75% (n = 21), and 57% (n = 16), respectively. Patients in age group 2 experienced the greatest mortality rate (75%) compared to age group 1 (9%) and age group 3 (22%). The calculated relative risk score indicated patients in age group 2 were 7.5 times more likely to die than other age groups combined (p = 0.02). CONCLUSIONS Primary ES of the spine is a rare, debilitating disease in which the role of surgery and its impact on one's quality of life and independence status has not been well described. This study found the majority of patients experienced a favorable outcome with respect to independence status following surgery and adjunctive treatment. An increased risk of recurrence and death was also present among the adolescent age group (14-20 years).