Abstract
1600 Background: Association of congenital disease (CD) and malignancy was noted in some studies. However, the cancer risk and patterns of different CD was unclear. In this study, we aimed to assess and quantify the relationships between CD and cancers. Methods: In the National Health System of Taiwan, 2.9 million children were born between 1998 and 2010. Patients with CD were identified using ICD-9-CM 740-759. Cox’s proportional hazards regression analysis was used to assess the cancer risk of CD. Results: A total of 32358 CD patients were identified and 87 of them developed malignancy. The cancer diagnostic age was significantly younger in patients with CD (2.44 ± 2.28 years) than patients without CD (3.97 ± 3.04 years; p<0.001). The hazard ratio (HR) of cancer incidence was 2.70 (95% confidential interval (CI) 2.12-3.44). Subgroup analysis showed that significantly increased risk of cancer development was noted among patients with cardiac anomalies (N=26, HR=1.91, 95% CI 1.28-2.86), digestive anomalies (N=8, HR=3.32, 95% CI 1.64-6.70), cutaneous anomalies (N=2, HR=21.5, 95% CI 5.35-86.40) and chromosomal anomalies (N=29, HR=17.5, 95% CI 12.00-25.70); other congenital diseases did not. Considering cancer types, patients with cardiac anomalies had higher rate of hematological cancers (N=12, HR=2.09, 95% CI. 1.15-3.79), especially acute myeloid leukemia (AML; N=9, HR=12.2, 95% CI. 5.36-28.0). Patients with digestive anomalies also had higher rate of hematological cancers (N=4, HR=3.95, 95% CI. 1.46-10.70), and the dominant type was non-Hodgkin lymphoma (N=2, HR=14.70, 95% CI. 3.38-64.0). Patients with chromosomal anomalies had superior rate to have retroperitoneal sarcoma (N=1, HR=8.54, 95% CI. 1.14-63.90), anterior mediastinal tumor (N=1, HR=8.54, 95% CI. 1.14-63.90), and hematological cancers (N=23, HR=33.20, 95% CI. 21.20-51.80), especially the AML (N=21, HR=231, 95% CI. 119-448). Down syndrome patients had extremely high risk of AML (N=8, HR=388, 95% CI. 164-918). Conclusions: Patients with cardiac, digestive, cutaneous and chromosomal CD were at increased risk and younger age to develop cancer. Hematological malignancies, especially AML, were the most common cancer type among these patients.
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