Recurrence of Ewing sarcomas of the chest wall

Ewing sarcomas (ES) of the chest wall are rare. Local recurrences occur in approximately 20% of these patients; however literature on this topic is scarce. Our aim was to analyze the influence of the extent of surgical resection on outcome, and to find positive prognostic factors for survival.

A retrospective analysis of all patients who were under 18 and treated between 1977 and 2006 for Ewing sarcoma of the chest wall was performed.

Twenty-seven patients with ES were diagnosed at our institution; 14 males and 13 females. Twenty-three patients underwent local resection, which was complete in 13. Nine patients had a wide resection, including more ribs than just the affected one. Complete remission (CR) was achieved in 14/27 patients. Recurrence was observed in 13 patients: local recurrence (LR) and distant metastasis (DM) both in 7. One of the patients had both LR and DM. All patients with LR and two patients with DM only died of disease. Overall survival after 10 and 20 years was 73% and 47%, respectively. Complete resection, wide resection, gender, or age were no significant factors affecting outcome.

Relapse carries a high mortality risk and none of the patients with LR eventually survived. Wide resection of at least a complete normal adjacent rib above and below the thoracic wall tumor as a fixed rule seems unnecessary.