New outlook on the diagnosis, treatment and follow-up of childhood-onset craniopharyngioma

The clinical, neuroradiological and surgical definition of hypothalamic involvement is a fundamental factor related to poor postoperative outcome, progressive obesity and neuropsychological impairment in children after surgical removal of craniopharyngiomaThe previously assumed 'gold-standard' objective of a primary radical removal of the lesion in all cases needs to be replaced with the new paradigm of a limited resection plus focused radiotherapy in patients with craniopharyngioma and hypothalamic lesionsHypothalamic involvement and treatment-related hypothalamic lesions are associated with the highest risk of postoperative sequelae and impaired quality of survival3D intensity-modulated proton beam radiotherapy has potential advantages over photon beam methods to focus and limit the radiation effects to optic and hypothalamic structuresPreclinical, in vivo mouse models can be used to investigate the pathogenesis of adamantinomatous craniopharyngioma and to test novel treatments