New Prognostic Indicators in Pediatric Adrenal Tumors: Neuroblastoma and Adrenal Cortical Tumors, Can We Predict When These Will Behave Badly?

Abstract

Pediatric adrenal tumors are unique entities with specific diagnostic, prognostic, and therapeutic challenges. The adrenal medulla gives rise to peripheral neuroblastic tumors (pNTs), pathologically defined by their architecture, stromal content, degree of differentiation, and mitotic-karyorrhectic index. Successful risk stratification of pNTs uses patient age, stage, tumor histology, and molecular/genetic aberrations. The adrenal cortex gives rise to adrenocortical tumors (ACTs), which present diagnostic and prognostic challenges. Histologic features that signify poor prognosis in adults can be meaningless in children, who have superior outcomes. The key clinical, pathologic, and molecular findings of pediatric ACTs have yet to be completely identified.

Keywords: ALK; Adrenocortical adenoma; Adrenocortical carcinoma; Ganglioneuroblastoma; Ganglioneuroma; MYCN; Neuroblastoma; TP53.