The risk of developing acute myeloid leukaemia in patients with Ewing’s sarcoma and trend analysis: A SEER-based study

Abstract Background Ewing sarcoma is a neoplasm of neuroectodermal origin arising from bone or soft tissue. The annual incidence of Ewing sarcoma is 2.93 children per 1,000,000. Acute myeloid leukaemia (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, our aim is to quantify the risk of developing AML in Ewing sarcoma patients and provide an updated evidence to the literature.Methods 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 the MP-SIR session to identify patients diagnosed with acute myeloid leukaemia as a second primary malignancy after Ewing sarcoma 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 P < 0.05.Results A total of 2631 patients with Ewing sarcoma were recorded in the SEER database, with a median follow-up of 120 + months. Patient with Ewing sarcoma had an increased risk to develop AML with O/E of 145.98 (95% CI:101.68 -203.03, P < 0.05, EAR = 21.79). The total cohort of patients had a PC of 32.7, APC of 1.3 (95% [CI] 0.6 -2.0) (P < 0.05). Female patients had a PC of 46.5, APC of 1.7 (95% [CI] 0.5–2.9; P < 0.05). Of relevance, 35 patients developed ANLL with O/E 130.92 (95% [CI] 91.19 -182.08; P < 0.05, EAR 21.77).Conclusion Patients treated for a primary Ewing sarcoma have significant high risk of adeveloping Acute myeloid leukaemia, among other second primary malignancies. We would recommend screening for AML from 2 to 11 months after beginning of Ewing sarcoma treatment.