Composite ganglioneuroblastoma. An assessment of the significance of histological maturation in neuroblastoma diagnosed beyond infancy

Abstract

At Cincinnati Childrens Hospital Medical Center, 19 cases of composite ganglioneuroblastoma (CGNB) comprise one third of the cases of neuroblastoma diagnosed after 1 year of age. We have not identified this condition in an infant. Usually located in the mediastinum, CGNB is typically resectable and has a favorable prognosis, but retains potential to metastasize until nests of neuroblasts have disappeared. Prognosis is independent of proportion of ganglioneuroma or neuroblastoma and is closely related to stage. The interval between diagnosis and death in fatal CGNB was typically prolonged. Survival of older children with poorly differentiated neuroblastoma was rare and neuromatous maturation was absent or trivial in unresected primary tumors studied at the time of autopsy. We conclude that favorable outcome in neuroblastoma diagnosed after 1 year of age is closely related to histological maturation as herein defined.