Childhood craniopharyngioma: treatment strategies and outcomes

Abstract

Craniopharyngiomas are embryogenic malformations of the sellar region. With an overall incidence of 0.5-2.0 new cases per million population per year, approximately 30-50% of all cases represent childhood craniopharyngioma. Therapy of choice in patients with favorable tumor localization is complete resection with a specific focus on maintaining functions of the optical nerve and hypothalamic-pituitary axes. In patients whose unfavorable tumor localization makes maintaining hypothalamic functionality surgically challenging, a limited resection followed by local irradiation is recommended. The overall survival rates are high (92%) but relapses and reduced QoL in survivors are also frequent. Because irradiation is efficient in preventing tumor progression, appropriate timing of post-surgical irradiation is currently under investigation in a randomized trial (KRANIOPHARYNGEOM 2007). Childhood craniopharyngioma should be recognized as a chronic disease requiring constant monitoring of the clinical and QoL consequences in order to provide optimal care of surviving patients.