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New Outlook On The Diagnosis, Treatment And Follow-up Of Childhood-onset Craniopharyngioma

H. Müller, T. Merchant, S. Puget, J. Martinez-Barbera
Published 2017 · Medicine

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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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10.1016/j.bbrc.2019.04.142
Frequent B7-H3 overexpression in craniopharyngioma.
C. Chen (2019)
10.1007/978-3-030-41176-3_10
Disease and Treatment-Related Hypothalamic Alterations in Craniopharyngioma: Clinical Presentation, Prognostic Impact, and Implications for Treatment Strategies
H. Müller (2020)
10.1002/pbc.28493
SIOP PODC-adapted treatment guidelines for craniopharyngioma in low- and middle-income settings.
Nisreen Amayiri (2020)
10.1530/ERC-18-0538
SHH pathway inhibition is protumourigenic in adamantinomatous craniopharyngioma
G. Carreno (2019)
10.1111/jne.12842
Oxytocin release deficit and social cognition in craniopharyngioma patients
Marie-Luise Brandi (2020)
10.3892/ol.2020.11309
Feasibility of primary human cell cultures as a model for adamantinomatous craniopharyngioma research: Evidence from RNA-Seq analysis
Pei-dong Zhang (2020)
10.1080/14737175.2018.1528874
Childhood-onset craniopharyngioma: latest insights into pathology, diagnostics, treatment, and follow-up
A. Bogusz (2018)
10.1016/j.wneu.2019.02.212
Immune Microenvironment of Primary and Recurrent Craniopharyngiomas: A Study of the Differences and Clinical Significance.
D. Lin (2019)
10.1007/s00381-020-04833-x
Management of pediatric craniopharyngioma: 10-year experience from high-flow center
Abd El Rahman Enayet (2020)
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