Müller, HL;
Merchant, TE;
Puget, S;
Martinez-Barbera, JP;
(2017)
New outlook on the diagnosis, treatment and follow-up of childhood-onset craniopharyngioma.
Nature Reviews Endocrinology
, 13
(5)
pp. 299-312.
10.1038/nrendo.2016.217.
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Abstract
Childhood-onset craniopharyngiomas are rare embryonic tumours of low-grade histological malignancy. Novel insights into the molecular pathogenesis of human adamantinomatous craniopharyngioma have started to unveil the possibility of testing novel treatments targeting pathogenic pathways. Hypothalamic involvement and/or treatment-related lesions result in impaired physical and social functionality and in severe neuroendocrine sequelae. Quality of survival in patients with craniopharyngioma with hypothalamic involvement is impaired by severe obesity, physical fatigue and non-optimal psychosocial development. Patients with craniopharyngioma involving hypothalamic structures have reduced 20-year overall survival, but overall and progression-free survival are not related to the degree of surgical resection. Irradiation is effective in the prevention of tumour progression and recurrence. For favourably localized craniopharyngiomas, the preferred treatment of choice is to attempt complete resection with preservation of visual, hypothalamic and pituitary function. For unfavourably localized tumours in close proximity to optic and/or hypothalamic structures, a radical neurosurgical strategy attempting complete resection is not recommended owing to potential severe sequelae. As expertise has been shown to have an impact on post-treatment morbidity, medical societies should establish criteria for adequate professional expertise for the treatment of craniopharyngioma. On the basis of these criteria, health authorities should organize the certification of centres of excellence that are authorized to treat and care for patients with this chronic disease.
| Type: | Article |
|---|---|
| Title: | New outlook on the diagnosis, treatment and follow-up of childhood-onset craniopharyngioma |
| Location: | England |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1038/nrendo.2016.217 |
| Publisher version: | http://dx.doi.org/10.1038/nrendo.2016.217 |
| Language: | English |
| Additional information: | Copyright © 2017 Macmillan Publishers Limited, part of Springer Nature. All rights reserved. The final publication is available at Nature Reviews Endocrinology via http://dx.doi.org/10.1038/nrendo.2016.217 |
| Keywords: | Hypothalamus, Neuroendocrine cancer, Neurosurgery, Obesity, Radiotherapy |
| UCL classification: | UCL 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 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/1541655 |
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