Resection of pulmonary metastases in pediatric patients with Ewing sarcoma improves survival

Abstract

Background: Ewing sarcoma (ES) is the second most common bone tumor in children, and survival of those with metastatic ES has not improved. Previous studies have shown a survival benefit to whole lung irradiation in patients with pulmonary metastases and may be given either before, after, or instead of surgical pulmonary metastasectomy (PM). The contribution of surgery compared with irradiation in ES has not previously been studied.

Methods: A retrospective review of patients younger than 21 years (median age, 16 years) treated at a single institution (1990-2006) was performed. Kaplan-Meier survival curves were compared using log-rank test and a multivariate Cox proportional hazards model. P ≤ .05 was regarded as significant.

Results: Eighty patients with ES were identified. Of these, 31 (39%) had pulmonary metastases. Nine patients had incomplete details of their full treatment regimen, but the following groups could be defined from the remainder: resection alone (n = 5), radiation alone (n = 3), radiation and resection (n = 3), or chemotherapy alone (n = 11). There were 24 deaths overall, with a median overall survival (OS) of 2.7 (95% confidence interval [CI], 1.7-5.2) years. Patients who had PM had the best OS (80%), whereas those who underwent radiation to the lung without PM compared with chemotherapy only for pulmonary metastasis both had similar OS of 0% at 5 years (P = .002). Patients who had radiation followed by PM for lung metastasis had a 5-year OS of 65%. Patients with PM had a longer OS compared with those without lung resection (P < .0001).

Conclusion: These data suggest a possible benefit for ES patients who undergo surgical resection of lung metastases.

Fig. 1

Kaplan-Meier curve of OS comparing ES patients who underwent surgical metastasectomy (n = 10) and those who did not (n = 21).

Fig. 2

Kaplan-Meier curve of RFS comparing ES patients who underwent surgical metastasectomy (n = 10) and those who did not (n = 21).

Fig. 3

Kaplan-Meier curve of OS comparing ES patients in 4 different groups: “radiation and resection” (n = 3), “radiation no resection” (n = 3), “resection no radiation” (n = 5), and “no resection no radiation” (n = 11).

Fig. 4

Kaplan-Meier curve of RFS comparing ES patients in 4 different groups: “radiation and resection” (n = 3), “radiation no resection” (n = 3), “resection no radiation” (n = 5), and “no resection no radiation” (n = 11).