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Adamantinomatous Craniopharyngioma: Genomics, Radiologic Findings, Clinical, and Prognosis

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Pituitary Disorders of Childhood

Part of the book series: Contemporary Endocrinology ((COE))

Abstract

Adamantinomatous craniopharyngiomas (ACPs) are histologically benign but clinically aggressive tumours due to their tendency to invade nearby structures, such as the hypothalamus or the optic pathways, and their recalcitrant tendency to relapse. Molecular and murine studies strongly suggest that ACPs are simple tumours initiated by mutations in β-catenin leading to the over-activation of the WNT pathway. ACPs, mostly in case of tumours with hypothalamic involvement, are associated with a high degree of morbidity and reduced 20-year overall survival. Quality of survival in patients with hypothalamic involvement is impaired by severe obesity, physical fatigue, severe neuroendocrine deficits, and nonoptimal psychosocial development. Current treatments include surgery followed by radiotherapy, but these are not always curative and can also be associated with high morbidity. Long-term follow-up studies suggest that for favourably localized tumours, the preferred treatment of choice is to attempt complete resection with preservation of visual, hypothalamic, and pituitary function, whilst incomplete resection is recommended for tumours with invasion of optic and/or hypothalamic structures. Hypothalamic obesity has a significant impact on long-term quality of survival and is the most frequent sequelae in patients with hypothalamic involvement. However, no therapy for ACP patients with hypothalamic obesity has been proven to be effective in randomized trials. In this chapter, we present recent findings on the biology of human ACP and discuss clinical data of relevance for the physicians managing ACP patients.

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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. H.L. Müller is supported by the German Childhood Cancer Foundation, Bonn, Germany. This work was supported by the Medical Research Council (MRC) (Grants MR/M000125/1), Great Ormond Street Hospital for Children Charity/Children with Cancer UK (GOSHCC/CWCUK) (Grant W1055), and National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children National Health Service Foundation Trust and University College London. J.P. Martinez-Barbera is a Great Ormond Street Hospital for Children’s Charity Principal Investigator.

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  1. Department of Pediatrics and Pediatric Hematology/Oncology, Klinikum Oldenburg AöR, Medical Campus University Oldenburg, Oldenburg, Germany

    Hermann L. Müller

  2. UCL Great Ormond Street Institute of Child Health, Developmental Biology and Cancer, Birth Defects Research Centre, London, UK

    Juan Pedro Martinez-Barbera

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  1. Division of Pediatric Endocrinology, New York University, New York, NY, USA

    Brenda Kohn

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Müller, H.L., Martinez-Barbera, J.P. (2019). Adamantinomatous Craniopharyngioma: Genomics, Radiologic Findings, Clinical, and Prognosis. In: Kohn, B. (eds) Pituitary Disorders of Childhood. Contemporary Endocrinology. Humana Press, Cham. https://doi.org/10.1007/978-3-030-11339-1_3

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