Primary Pediatric Brain Tumors of the Posterior Fossa: Part I

Abstract

In pediatric neuro-oncology practice, cerebellar tumors are often referred to as infratentorial tumors or tumors of the posterior fossa (a differential diagnosis is provided in Table 1). This anatomic region also contains the pons and medulla, which along with the midbrain comprise the brainstem. In Part I of this comprehensive review, three important pediatric brain tumors usually localized to the cerebellum are discussed (and summarized in Table 2): atypical teratoid/rhabdoid tumors (ATRTs), pilocytic astrocytomas, and ependymomas. In the companion chapter (Part II), an integrated clinical and molecular overview of medulloblastoma follows. These tumors have been selected, in part, due to their clinical significance as well as recent advances in their molecular genetics and pathological classification. For these entities and others, the histopathologic, cytogenetic, and molecular factors have been integrated into the updated fourth edition of the World Health Organization (WHO) Classification of Tumors of the Central Nervous System (Louis et al., WHO classification of tumours of the central nervous system, Revised 4th edn. IARC, Lyon, 2016a; Louis et al., Acta Neuropathol 131:803–820, 2016b).