The risk of developing acute myeloid leukemia in patients with Ewing sarcoma and trend analysis: a SEER-based study

Ewing sarcoma (ES) is a neoplasm of neuroectodermal origin arising from bone or soft tissue. The annual incidence of ES is 2.93 per 1, 000, 000. Acute myeloid leukemia (AML) is one of the most described second malignancies as a complication of primary cancer therapy. There is a lack of recent studies elaborating on the incidence rates of such complications. So, the aim was to quantify the risk of developing AML as a second primary malignancy (SPM) in ES patients and to provide an updated evidence to the literature.

We extracted the data from the Surveillance, Epidemiology and End Results (SEER) program statistical analysis software package (SEER*Stat, version 8.4.1.2). We used an MP-SIR session to identify patients diagnosed with AML as an SPM after ES as a first primary malignancy between 2000 and 2020. We assessed the SIR as Observed/Expected(O/E) and Excess Absolute Risk (EAR) per 10, 000 with a 95% confidence interval (CI) and statistical significance at 0.05.

A total of 2631 patients with ES were recorded in the SEER database, with a median follow-up of 120 + months. Patients with ES had an increased risk of developing AML with an O/E of 145.98 (P < 0.05, EAR = 21.79). Gender played a role in AL development; both males (O/E = 52.94, P < 0.05, 95% CI: 31.87-82.670) and females (O/E = 105.62, P < 0.05) had a high risk of AL SPM. About 35 patients developed acute non-lymphocytic leukemia with an O/E 130.92 (P < 0.05, 95% CI: 91.19-182.08, EAR 21.77). There was a significantly increased risk of developing SPMs in different sites among ES patients (O/E = 5.85, P < 0.05).

Patients treated for a primary ES have a significant risk of developing AML, among other second primary malignancies. Thus, we recommend screening for AML from 2 to 11 months after the diagnosis of ES for early detection and better management outcomes.