Abstract
Over the last few years, there have been significant advances in the understanding of the pathogenesis of human adamantinomatous craniopharyngioma. Global and gene-specific expression studies of human biopsies have helped to delineate the network of pathways involved in the initiation and establishment of these tumours. Interestingly, genetically modified and patient-derived xenograft mouse models have revealed the function of some of the dysregulated pathways as well as identified a role for specific tumour cells in the formation of these neoplasias. It has emerged that undifferentiated embryonic precursors and adult pituitary stem cells are critical in the pathogenesis of murine tumours resembling human ACP. In this chapter, we aim to provide a general overview of pituitary development, since there is strong case for a developmental origin for human ACP. We also discuss the in vitro and in vivo evidence demonstrating the presence of stem cells inthe adult pituitary as well as their role of the pathogenesis of craniopharyngioma.
| Original language | English |
|---|---|
| Title of host publication | Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma |
| Publisher | Springer International Publishing Switzerland |
| Pages | 41-55 |
| Number of pages | 15 |
| ISBN (Electronic) | 9783319518909 |
| ISBN (Print) | 9783319518886 |
| DOIs | |
| Publication status | Published - 19 Apr 2017 |
Keywords
- Craniopharyngioma
- Mouse models
- Pituitary
- Sox2
- Stem cells
- WNT pathway