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Molecular and cellular pathogenesis of adamantinomatous craniopharyngioma.

Martinez-Barbera, JP; (2015) Molecular and cellular pathogenesis of adamantinomatous craniopharyngioma. Neuropathol Appl Neurobiol 10.1111/nan.12226. (In press). Green open access

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Abstract

Adamantinomatous craniopharyngiomas (ACPs) are the most common pituitary tumours in children. Although histologically benign, these are clinically aggressive tumours, difficult to manage and associated with poor quality of life for the patients. Several human and mouse studies have provided unequivocal evidence that the over-activation of the WNT/β-catenin signalling pathway underlies the molecular aetiology of these tumours. Recently, research using genetically modified mouse models of human ACP have revealed a critical and unexpected non-cell autonomous role for pituitary stem cells in ACP tumourigenesis, which has expanded the cancer stem cell paradigm. As the result of this basic research, the pathogenesis of ACP is being unveiled, with promising implications for the development of novel treatments against these childhood neoplasms. These benign tumours may additionally represent a unique model to provide insights into the initial steps of oncogenesis.

Type: Article
Title: Molecular and cellular pathogenesis of adamantinomatous craniopharyngioma.
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/nan.12226
Publisher version: http://dx.doi.org/10.1111/nan.12226
Additional information: © 2015 The Authors. Neuropathology and Applied Neurobiology published by John Wiley & Sons Ltd on behalf of British Neuropathological Society. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Keywords: Sox2, WNT pathway, adamantinomatous craniopharyngioma, pituitary, stem cells, β-catenin
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Biology and Cancer Dept
URI: https://discovery.ucl.ac.uk/id/eprint/1467365
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