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

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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).


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).


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).


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).

ABBREVIATIONS ES = Ewing sarcoma; FSH = follicle stimulating hormone; IGF-1 = insulin-like growth factor 1; LH = luteinizing hormone; mRS = modified Rankin Scale; PRISMA = Preferred Reporting Items for Systematic Reviews and Meta-Analyses; PTH = parathyroid hormone; QoL = quality of life; RR = relative risk.

Article Information

Correspondence R. John Hurlbert: University of Arizona, Tucson, AZ. rjhurlbert@surgery.arizona.edu.

INCLUDE WHEN CITING Published online February 15, 2019; DOI: 10.3171/2018.10.SPINE18795.

G.K.B. and P.L.N. contributed equally to this work.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.



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    PRISMA diagram. Figure is available in color online only.

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    Bar graph showing the prevalence of ES by age. Figure is available in color online only.

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    Bar graph of patient mortality by age group. * Statistically significant. Figure is available in color online only.

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    Hormone changes by age. This figure illustrates the findings regarding known hormone levels spanning several studies from the literature. Each figure diagrams a surge in hormone levels at or near the time of life when the transition of prepubescent to pubescence would typically be expected to occur. A: FSH and LH. Data gathered from Burr et al. 1970 and Rosenfield et al. 2013.11,56 B: PTH. Data gathered from Cioffi et al. 2000.12 C: IGF-1. Data gathered from Alberti et al. 2011.4 D: Estradiol (E2) and total testosterone (Test). Data gathered from Dimitrakakis et al. 2002, Garcia-Mayor et al. 1997, and Kelsey et al. 2014.19,24,34 The y-axis on the left shows E2 levels while that on the right represents Test levels. In the case of male Test, units were converted from nmol/L (cited in the original manuscript) to pg/ml in order to match the unit types reported in the other studies. Figure is available in color online only.





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