Background: Patients are at risk of second malignancies (SM) after treatment for Ewing sarcoma family of tumours (ESFT).
Methods: We performed a retrospective review of 237 patients with ESFT treated at our institution from September 1979 through to February 2004. Cumulative incidence (CI) of SM by the type of malignancy and treatment was estimated.
Results: Twelve patients with SM were identified. Secondary leukaemia (SL) developed in 8 patients (2 ALL, 6 MDS/AML), a median 2.6 years (range 1.4-19.6 years) after diagnosis of ESFT. Four patients had secondary solid tumours, a median 8.0 years (range 7.4-9.4 years) after the ESFT diagnosis. Five- and 10-year estimates of the CI of SM were 3.0+/-1.1% and 4.7+/-1.5%, respectively. Patients treated on recent protocols with higher cumulative doses or an increased dose intensity of alkylators and epipodophyllotoxins and the use of G-CSF had a higher estimated CI of SL than those in earlier studies (5-year CI 6.4+/-2.4% versus 0.0+/-0.0%, respectively, P=0.004).
Conclusions: Patients with ESFT are at risk for SM after treatment. The cumulative incidence of SM is higher with the current treatment protocols and may be related to the intensification of chemotherapeutic agents.