Rare Embryonal Brain Tumours

Abstract

Rare embryonal brain tumors of childhood include a wide spectrum of diseases, including tumors historically called central nervous system/supratentorial primitive neuroectodermal tumors (CNS-PNETs/SPNET), pineoblastomas (PB), atypical teratoid rhabdoid tumors (AT/RTs), and more recently described entities such as embryonal tumor with multilayered rosettes (ETMRs). Although these rare tumors are generally collectively considered to represent more aggressive, treatment-resistant diseases arising in younger children, the clinicopathologic and biological features of these rare diseases are only beginning to be elucidated. Cumulative global collaborative efforts over the last 15 years have led to a clearer understanding of the histogenesis and molecular spectra of these tumors. In this chapter, we will review recent developments in biological understanding of these rare embryonal brain tumors and the prospects for novel therapeutic approaches and improved clinical management.