2013
DOI: 10.1007/s00381-013-2309-z
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Familial syndromes associated with intracranial tumours: a review

Abstract: This review examines syndromes that are strongly associated with CNS cancers: the phakomatosis syndromes, familial syndromes such as Li-Fraumeni and familial polyposis syndromes and dyschondroplasia.

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Cited by 26 publications
(7 citation statements)
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“…Due to its low incidence it is difficult to assess the role of environmental and genetic factors, and also the possible interactions among them. Some hereditary syndromes increase the risk of these tumors, such as such as neurofibromatosis, Li-Fraumeni syndrome, tuberous sclerosis, Von Hippel-Lindau syndrome or retinoblastoma 3 , 4 .…”
Section: Introductionmentioning
confidence: 99%
“…Due to its low incidence it is difficult to assess the role of environmental and genetic factors, and also the possible interactions among them. Some hereditary syndromes increase the risk of these tumors, such as such as neurofibromatosis, Li-Fraumeni syndrome, tuberous sclerosis, Von Hippel-Lindau syndrome or retinoblastoma 3 , 4 .…”
Section: Introductionmentioning
confidence: 99%
“…Trainees should be familiar with hereditary disorders that predispose to central nervous system tumors. 5 -8 These can be accomplished at all types of training programs by incorporating a didactic lecture series as well as expanding the residents’ clinical experiences by providing increased exposure to this patient population.…”
Section: Proposed Approaches To Incorporate Training In Neuro-oncologmentioning
confidence: 99%
“…As highlighted above, associations have also been established between gliomas and several rare inheritable syndromes, such as Phakomatoses (neurocutaneous syndromes), Li-Fraumeni, enchondromatosis, and familial polyposis syndromes, which account for <5% of all gliomas ( 16 ). Variability in glioma incidence was noted between different ethnic origins, with Caucasian individuals being at higher risk of developing a malignant CNS tumor compared to African-Americans, Asian and Asian/Pacific Islanders ( 17 ).…”
Section: Introductionmentioning
confidence: 99%