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Pathogenesis of Human ACP

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Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma

Abstract

In 2002 J.T. Rutka stated in an editorial commentary that: “There is perhaps no other primary brain tumour that evokes more passion, emotion, and, as a result, controversy than does the craniopharyngioma”. This statement clarifies the situation of medical specialists such as endocrinologists, neurosurgeons, neuropathologists, neurooncologists, paediatric oncologists and neuro-radiologists involved in treatment of craniopharyngioma (CP) patients. Although CPs are classified by the WHO as benign tumours, their size and relationship to multiple critical structures can incur massive clinical complications. Here we elucidate the histological and genomic hallmarks, setting adamantinomatous (ACP) apart from papillary craniopharyngiomas (PCP). In addition, molecular mechanisms that we already know to be involved in the pathogenesis of ACP, e.g. Wnt, SHH and EGFR signalling influencing proliferation, morphology, migration and recurrence by the establishment of a presumably tumour stem cell niche, are discussed. Our understanding of the driving force of ACP tumourigenesis opens new avenues for targeted treatment options, e.g. the inhibition of the EGFR.

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Authors and Affiliations

  1. University Hospital, Friedrich—Alexander University Erlangen-Nürnberg (FAU), Erlangen, Germany

    Annett Hölsken

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  1. Annett Hölsken
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Correspondence to Annett Hölsken .

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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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Hölsken, A. (2017). Pathogenesis of Human ACP. 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_1

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  • DOI: https://doi.org/10.1007/978-3-319-51890-9_1

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