neuroblastoma, IV-S syndrome

neuroblastoma, IV-S syndrome

Pediatric oncology A type of neuroblastoma (S for special) comprising 10-20% of all neuroblastomas, in which the primary tumor may be small, confined to the adrenal gland, but has widespread disease with massive involvement of the liver and skin; bone may be involved but osteolysis is not present; despite metastases, the tumor regresses spontaneously by maturation sequence neuroblastoma, the most immature lesion composed of neuroblasts, to ganglioneuroblastoma, finally to ganglioneuroma–the most mature of the sequence, composed of ganglion cells. See One-hit/two-hit theory.