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  • 1
    ISSN: 1432-1920
    Keywords: Key words Neuroepithelial tumor of childhood ; supratentorial ; Desmoplastic astrocytoma ; Desmoplastic ganglioglioma ; Magnetic resonance imaging ; Computed tomography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Desmoplastic neuroepithelial tumours are rare supratentorial neoplasms of infancy with a favourable prognosis. We characterised their imaging findings by reviewing the clinical and radiological reports of five affected children. The tumours were massive, predominantly cystic and with preferential frontal and parietal lobe involvement. The cystic component was in the white matter, with no communication with the ventricle. The solid part was superficially, abutting a meningeal surface, and showed intense contrast enhancement. A heterogeneous predominantly solid mass was observed in one patient. Thinning and deformation of the skull adjacent to the tumour was shown in four cases. Peritumoral oedema was absent or moderate. No calcification or haemorrhage were present. Angiography showed a tumour blush from the internal or external carotid arteries in one case each. Follow-up (3 months–9 years, mean 4.5 years) showed no recurrence.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1920
    Keywords: Neuroepithelial tumor of childhood, supratentorial ; Desmoplastic astrocytoma ; Desmoplastic ganglioglioma ; Magnetic resonance imaging ; Computed tomography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Desmoplastic neuroepithelial tumours are rare supratentorial neoplasms of infancy with a favourable prognosis. We characterised their imaging findings by reviewing the clinical and radiological reports of five affected children. The tumours were massive, predominantly cystic and with preferential frontal and parietal lobe involvement. The cystic component was in the white matter, with no communication with the ventricle. The solid part was superficially, abutting a meningeal surface, and showed intense contrast enhancement. A heterogeneous predominantly solid mass was observed in one patient. Thinning and deformation of the skull adjacent to the tumour was shown in four cases. Peritumoral oedema was absent or moderate. No calcification or haemorrhage were present. Angiography showed a tumour blush from the internal or external carotid arteries in one case each. Follow-up (3 months-9 years, mean 4.5 years) showed no recurrence.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 15 (1999), S. 676-680 
    ISSN: 1433-0350
    Keywords: Key words Craniosynostosis ; Genetics ; FGFR ; Msx2 ; Development ; Skull
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The genetic studies of syndromic craniosynostoses lead to the characterisation of genes that regulate the correct development of the bones of the skull. From these studies, it appears that FGF/FGFR signalling has a crucial role in this problem. Numerous mutations affecting the genes coding for FGFR1, 2 or 3 are responsible for these syndromes. It is interesting to note that some identical mutations produced various different phenotypes, suggesting that other genes modulate the phenotypic expressivity. The other involved genes in these syndromes code for such proteins as Msx2 or Twist that interact in the cellular pathways responsible for FGF action. From these genetic studies, it is now important to establish the role of these proteins during the development of the skull. Msx2 plays a repressive role in osteogenesis, whereas FGFRs act as promoting proteins. In the near future, it will be very important to improve our understanding of these phenomena in order to test specific treatments to prevent the development of such syndromes.
    Type of Medium: Electronic Resource
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