Adamantinomatous Craniopharyngioma (ACP) is a highly debilitating paediatric brain tumour that lacks medical anti-tumour therapies. Current therapy, which depends largely on surgery and radiation, is associated with poor quality of life and becomes more challenging and risky in the setting of recurrent disease. Recent discoveries regarding the biological characteristics of ACP indicate that available agents, including Mitogen-activated protein kinase/extracellular signal-regulated kinase (MAPK/ERK) pathway inhibitors may be effective in the control of ACP. Binimetinib is one such agent.

In this study, up to 38 patients will receive oral medication binimetinib for up to two years. The study will investigate if binimetinib will be safe and effective at inducing tumour response in children with residual ACP.