Association between congenital cardiovascular malformations and neuroblastoma.

OBJECTIVE: We explored the association between neuroblastoma and congenital cardiovascular malformations (CCM), previously described in case reports. STUDY DESIGN: Echocardiogram and chart reviews of a series of 158 patients with neuroblastoma and a control group of 192 children with leukemia were performed. The proportion of patients with CCM in each group was compared. RESULTS: Fourteen of the 70 (20%) patients with neuroblastoma and echocardiography had CCM, compared with 7 of the 192 (3.6%) patients with leukemia with echocardiograms (P=.0001). If all of the patients with neuroblastoma without echocardiograms (n=88) are considered to have normal cardiac anatomy, this difference remains significant (14 of 158 patients with neuroblastoma have CCM detected [8.9%] versus 7 of 192 patients with leukemia [3.6%]; P=.045). Neural crest-derived CCM were more common in patients with neuroblastoma, detected in 5 of 70 patients with neuroblastoma versus 2 of 192 patients with leukemia (P=.016). Congenital cardiovascular malformations in patients with neuroblastoma were associated with a cancer diagnosis at age less than 1 year and a lower neuroblastoma stage, but there was no association with tumor MYCN amplification. CONCLUSIONS: Neuroblastoma and CCM may be associated. We recommend echocardiography for CCM screening in patients with newly diagnosed neuroblastoma.