Abstract 277: A retrospective analysis of pediatric and adolescent oncology patients and congenital anomalies
Authors: Wong-Siegel Jeannette R. , Johnson Kimberly , Gettinger Katie , Druley Todd 
Published: July 2017

Abstract
Introduction: Congenital anomalies (CAs) are a leading cause of infant death and contribute to long-term disability and repeated hospitalizations. Many epidemiology studies have suggested that children with CAs have an increased cancer risk compared to those without CAs. This retrospective case series further investigates associations between CAs and cancer in a large academic center’s pediatric and adolescent oncology patient population.
Methods: Electronic medical records of 1,435 oncology patients diagnosed from birth to 23 years of age at St. Louis Children’s Hospital from January 1, 2004 - December 31, 2014 were reviewed. CA information was extracted and verified with ICD-9 codes when available. Patients followed for &lt;1 year at SLCH (n=193), and those diagnosed with a chromosomal anomaly or known cancer predisposition syndrome (n=113) were excluded from the analysis. Bivariate analyses compared demographic and other characteristics between patients with and without a CA with significant differences determined by chi-square tests. We calculated age-adjusted standardized rate ratios (SRRs) to evaluate whether the observed number of CAs among specific cancer types (benign, bone, CNS, epithelial, leukemia, lymphoma, germ cell, and soft tissue tumors) varied from the expected number using the CA rates from the entire cohort as the reference. Differences in survival time distributions in cancer cases with CAs versus those without CAs were evaluated with the log-rank test.
Results: Of 1,129 SLCH pediatric cancer patients, 156 (14%) patients were identified with a CA. Overall there was an increased proportion of patients with a CNS tumor who also had a CA compared to those without a CA (p=0.005). Neurological anomalies were specifically found to be in excess in CNS tumor cases versus the overall population of pediatric cancer patients (SRR= 1.42 95% CI 1.02-1.92 p=0.038). There were no significant differences by age at primary tumor diagnosis, but patients with a CNS tumor and CA were diagnosed an average of 1.5 years earlier (7.7 vs. 9.2 years, p=0.075) compared to those without a CA. The rate of CAs did not vary significantly by sex, but a significant excess of males with a neurological anomaly was observed among all patients diagnosed &lt;5 years of age (M/F ratio=2.55 95% CI 1.15-5.56 p=0.02). Finally, survival between those with and without a CA was not significantly different (p=0.24).
Conclusions: This study provides additional insight into the association between specific types of CAs and cancer development. Our results suggest children with neurological anomalies are more likely to develop CNS cancers and may be more likely to develop cancer at an earlier age, particularly in males. Our study supports the need for additional longitudinal surveillance and research that may improve outcomes as well as translational research to investigate any associated developmental mechanisms that may underlie tumor predisposition.
Citation Format: Jeannette R. Wong-Siegel, Kimberly Johnson, Katie Gettinger, Todd Druley. A retrospective analysis of pediatric and adolescent oncology patients and congenital anomalies [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2017; 2017 Apr 1-5; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2017;77(13 Suppl):Abstract nr 277. doi:10.1158/1538-7445.AM2017-277