| Popis: |
Embryonal tumours account for 20% of paediatric central nervous system (CNS) tumours. Medulloblastoma (MB), the most frequent, arises in the cerebellum. Clinical strategies have been based on series of multi-institutional trials since the 1970s. Recent understanding of the influence of molecular/biological factors has led to subdivision into four distinct subtypes with differing clinical and prognostic profiles, on which stratification is now based. Management of MB in adults is largely based on principles of managing children, modified according to differing clinical and toxicity profiles. Molecular analysis has led to the understanding that what was previously referred to as CNS-PNET comprise a heterogeneous group of tumours with a significant proportion representing other histologies. Atypical teratoid/rhabdoid tumour (AT/RT) carries mutations in the gene hSNF5/INI1 in most cases, with many now associated with an improved prospect of long-term survival. Pineal tumours have similar clinical presentations, comprising a heterogeneous mix of histologies from pineocytoma through to pineoblastoma. |
