Molecular and cellular pathogenesis of adamantinomatous craniopharyngioma

Adamantinomatous craniopharyngiomas (ACPs) are the most common pituitary tumours in children. Although histologically benign, these are clinically aggressive tumours, difficult to manage and associated with poor quality of life for the patients. Several human and mouse studies have provided unequivocal evidence that the over-activation of the WNT/β-catenin signalling pathway underlies the molecular aetiology of these tumours. Recently, research using genetically modified mouse models of human ACP have revealed a critical and unexpected non-cell autonomous role for pituitary stem cells in ACP tumourigenesis, which has expanded the cancer stem cell paradigm. As the result of this basic research, the pathogenesis of ACP is being unveiled, with promising implications for the development of novel treatments against these childhood neoplasms. These benign tumours may additionally represent a unique model to provide insights into the initial steps of oncogenesis.