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Nat Rev Endocrinol. 2017 May;13(5):299-312. doi: 10.1038/nrendo.2016.217. Epub 2017 Feb 3.

New outlook on the diagnosis, treatment and follow-up of childhood-onset craniopharyngioma.

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Department of Pediatrics and Pediatric Hematology and Oncology, Klinikum Oldenburg AöR, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, Germany.
Division of Radiation Oncology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, Tennessee 38105-3678, USA.
Service de Neurochirurgie, Hôpital Necker-Enfants Malades, Sorbonne Paris Cité, 149 Rue de Sèvres, 75015 Paris, France.
Developmental Biology and Cancer Programme, Birth Defects Research Centre, University College London (UCL) Great Ormond Street Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK.


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.

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