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Long-Term Management and Clinical Trials in Adamantinomatous Craniopharyngioma

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Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma

Abstract

This report is a review of findings on the diagnosis, treatment, clinical course, follow-up, and prognosis of craniopharyngioma patients with special regard to clinical trials and long-term management. Craniopharyngiomas are rare, embryonic malformations of the sellar/parasellar region with low histological grade. Clinical manifestations are related to increased intracranial pressure, visual impairment, and hypothalamic/pituitary deficiencies. If the tumour is favourably localised, therapy of choice is complete resection, with care taken to preserve hypothalamic and optic functions. In patients with unfavourable tumour location (i.e. involvement of hypothalamic areas), recommended therapy is limited hypothalamus-sparing surgical strategy followed by irradiation. Irradiation has proven effective in treatment of recurrences and progression. Surgical lesions and/or anatomical involvement of posterior hypothalamic areas can result in serious sequelae, mainly hypothalamic syndrome. It is crucial that craniopharyngioma can be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients by experienced multidisciplinary teams in the context of multicentre trials.

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Financial Disclosure

This manuscript was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author is supported by the German Childhood Cancer Foundation, Bonn, Germany.

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  1. Department of Pediatrics and Pediatric Hematology and Oncology, Klinikum Oldenburg AöR, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133, Oldenburg, Germany

    Hermann L. Müller

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  1. Developmental Biology and Cancer Programme, Birth Defects Research Centre, Institute of Child Health, University College London, London, United Kingdom

    Juan Pedro Martinez-Barbera

  2. Craniofacial Development and Stem Cell Biology, King’s College London, London, United Kingdom

    Cynthia Lilian Andoniadou

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Müller, H.L. (2017). Long-Term Management and Clinical Trials in Adamantinomatous Craniopharyngioma. In: Martinez-Barbera, J., Lilian Andoniadou, C. (eds) Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma. Springer, Cham. https://doi.org/10.1007/978-3-319-51890-9_10

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