Rare Embryonal Brain Tumours of Childhood

Summary

Embryonal brain tumours of childhood encompass a spectrum of histologic entities that are commonly grouped together as primitive neuroectodermal tumors (PNETs) or “small round blue cell tumours” of the Central Nervous System (CNS) and include medulloblastoma, CNS-PNETs and atypical teratoid/rhabdoid tumors (ATRTs). In contrast to medulloblastoma, the biology of CNSPNETs and ATRTs has remained poorly elucidated as the rarity of these tumour entities have limited robust biological and clinical studies and precluded the development of rational therapeutic studies. However recent large based consortia genomic and epigenomic studies, have led to the discovery of new diagnostic categories of PNETs and sub-types of ATRTs that have significant implications for future directions of clinical trials. In this talk, Dr Huang will review the most recent biological data from studies of PNETs and ATRTs.