Neuroblastoma and adrenal morphologic features in anencephalic infants.

At autopsy, an incidental congenital neuroblastoma was found in the adrenal gland of an 8-day-old full-term anencephalic baby. This previously unreported coincidence of neuroblastoma and anencephaly prompted a retrospective study of adrenal morphologic features in 20 anencephalic patients and 20 matched controls. Anencephalic adrenal glands were smaller and the fasciculoreticular zone was thinner (average 32%) than in the normal controls (average 76%). The neural crest-derived cells represented an average of 2% of the total gland area in anencephalic patients and 0.5% in the controls. These findings, statistically significant and compatible with previous studies, verify the deficient development of the fetal cortex in anencephalic patients. Furthermore, the neural component in anencephalic patients may be delayed in its maturation by the abnormal cortex, and thus may be prone to undergo malignant transformation and become a neuroblastoma.