Congenital tumors of the central nervous system

Congenital tumors of the central nervous system are rare but have a poor prognosis due to their size and location as well as the contraindication of radiotherapy at this age. Various classifications have been described based on the age of the patient at clinical manifestation. The short latency between tumor onset and clinical signs suggests a genetic predisposition, as has been described for some hereditary tumor syndromes that manifest in infancy (astrocytoma in tuberous sclerosis, choroid plexus tumor in Li Fraumeni syndrome, medulloblastoma in Gorlin syndrome, atypical rhabdoid/teratoid tumor in rhabdoid tumor predisposition syndrome). Here, we describe the clinical signs, pre- and postnatal diagnostic paradigms, histopathological and genetic characterization of the most frequent congenital tumors of the central nervous system: teratomas, astrocytomas, choroid plexus papillomas, primitive neuroectodermal tumors, medulloblastomas, atypical rhabdoid/terotoid tumors, craniopharyngiomas. Treatment options and pitfalls will be discussed. © 2011 Nova Science Publishers, Inc. All rights reserved.