Number and incidence of (major) malignancies and survival in the FANCD1/BRCA2 group of patients. (A) Pie chart depicting the number and occurrence of malignancies that patients (total n = 75) developed during their lives. (B) Cumulative probability of any (first) malignancy (median 1.9 years; 95% CI = 1.3–3.0). (C) Kaplan–Meier estimation of OS—median 3.8 years (95% CI = 2.8–5.0). (D) Probability of first diagnosis of specific malignancy (grouped into—peripheral embryonal tumors median 1.0 years of age, hematologic malignancies—1.8 and CNS tumors 2.7 years) in the FA-D1 group; embryonal tumors versus CNS log-rank test p < 0.05. (E) Influence of treatment on cumulative survival after the diagnosis of the malignant disease [median OS treated group 1.5 (95% CI = 0.9–2.7) vs. untreated 0.3 (95% CI = 0.1–1.2); p < 0.001]. In (B–E), the number of individuals with particular features at relevant time points is indicated below the graphs.
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