Adesina Adekunle M. - Atlas of Pediatric Brain Tumors
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- Year:2016;2018
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- In this second edition of the Atlas of Pediatric Brain Tumors , we have classified the tumor types based on the 2007 World Health Organization (WHO) classification of brain tumors (Table ). We have also incorporated revisions in the new 2016 WHO classification of brain tumors, with emphasis on the molecular stratification of these tumors.Table 1.1Classification of tumors of the nervous systemAstrocytic TumorsPilocytic astrocytomaPilomyxoid astrocytomaPleomorphic xanthoastrocytomaDiffuse astrocytomaAnaplastic astrocytomaGlioblastomaGiant cell glioblastomaGliosarcomaGlioblastoma cerebriOligodendroglial TumorsOligodendrogliomaAnaplastic oligodendrogliomaOligoastrocytomaAnaplastic oligoastrocytomaEpendymal TumorsEpendymomaAnaplastic ependymomaMyxopapillary ependymomaSubependymomaChoroid Plexus TumorsChoroid plexus tumorsOther Neuroepithelial TumorsAstroblastomaChordoid glioma of the third ventricleAngiocentric gliomaNeuronal and Mixed Neuronal-Glial TumorsGanglioglioma and gangliocytomaDesmoplastic infantile astrocytoma and gangliogliomaCentral neurocytoma and extraventricular neurocytomaCerebellar liponeurocytomaPapillary glioneuronal tumor (PGNT)Rosette-forming glioneuronal tumor of the fourth ventricle (RGNT)Paraganglioma (spinal)Tumors of the Pineal RegionPineocytomaPineal parenchymal tumor of intermediate differentiationPineoblastomaPapillary tumor of the pineal regionEmbryonal TumorsMedulloblastomaCNS Primitive neuroectodermal tumor (PNET)MedulloepitheliomaEpendymoblastomaAtypical teratoid/rhabdoid tumorTumors of the Cranial NervesSchwannomaNeurofibromaPerineuriomaMalignant peripheral nerve sheath tumor (MPNST)Meningeal TumorsMeningiomasMesenchymal, non-meningothelial tumorsHemangiopericytomaMelanocytic lesionsHemangioblastomaTumors of the Hematopoietic SystemMalignant lymphomasHistiocytic tumorsGerm Cell TumorsCNS germ cell tumorsFamilial Tumor SyndromeNeurofibromatosis type 1Neurofibromatosis type 2SchwannomatosisVon Hippel-Lindau disease and hemangioblastomaTuberous sclerosis complex and subependymal giant cell astrocytomaLi-Fraumeni syndrome and TP53 germline mutationsCowden disease and dysplastic gangliocytoma of the cerebellum/Lhermitte-Duclos diseaseTurcot syndromeNevoid basal cell carcinoma syndromeRhabdoid tumor predisposition syndromeTumors of the Sellar RegionCraniopharyngiomaGranular cell tumor of the neurohypophysisPituicytomaSpindle cell oncocytoma of the adenohypophysisMetastatic Tumors of the CNS
- The WHO classification assumes that the pattern of differentiation reflects the cell of origin.
- The long-standing concept suggesting that brain tumors arise as a result of degeneration of mature cell types has been questioned.
- Current concepts suggest that all tumors arise from cancer stem cells, which in the central nervous system (CNS) share some common characteristics with neural progenitor stem cells, including:
- Ability for self-renewal
- Ability for divergent differentiation
- The extent or pattern of differentiation reflects the nature of activation and deregulation of specific signaling pathways.
- Deregulation is often directed at genes that control nodal points where cell growth, proliferation, and differentiation converge with the upregulation of genes involved in organismal survival and deregulation of cell cycle control.
- The WHO grading system is a four-tier grade system with each increasing grade implying lesser degrees of differentiation, increasing anaplasia, and increasing proliferative potential and mitotic activity.
- The higher-grade tumors acquire aggressive characteristics, which in the astrocytomas include vascular proliferation and necrosis.
- The proportion of tumor cells in the cancer stem cell pool is lowest in the lower-grade tumors and highest in the higher-grade tumors; it correlates with the proliferation indices of these tumors.
- In the astrocytomas, tumors with a discrete growth pattern rather than an infiltrative growth pattern are often amenable to surgical treatment with good survival, and are often graded as Grade I (e.g., pilocytic astrocytoma).
- Increasing anaplasia is associated with clonal evolution of a subpopulation of cells that have acquired additional genetic events (e.g., myc amplification), resulting in transformation of classic medulloblastomas to anaplastic/large-cell medulloblastomas.
- Although a tissue-based diagnosis must be made and must stand by itself independent of neuroradiologic findings, the presence of concordance or correlation between the surgical pathology impression and the neuroradiologic differential diagnoses is very reassuring.
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