Peripheral neuroblastic tumors of the adrenal gland: clinicopathologic features and important molecular alterations

Peripheral neuroblastic tumors are the most common extracranial solid tumor of childhood and commonly present as an adrenal mass in a young child. This group of tumors is notable for wide clinical heterogeneity and variable clinical outcome. Peripheral neuroblastic tumors are defined by the amount of Schwannian stroma and degree of neuroblastic differentiation and categorized by the International Pathology Neuroblastoma Committee (INPC) as neuroblastoma, ganglioneuroblastoma and ganglioneuroma. Molecular testing remains critical for risk stratification of patients. Important prognostic factors include age, stage, histologic classification, and amplification of MYCN. Advancements in our understanding of clinical and biologic prognostic factors continue to provide valuable knowledge for risk stratification and potential therapeutic targets.