Shift towards less radical, hypothalamus-sparing treatment strategies in childhood craniopharyngioma – Comparison between patients recruited in KRANIOPHARYNGEOM 2000 and 2007

Abstract

Background: Despite high survival rates in childhood craniopharyngioma, prognosis is frequently impaired due to sequelae. Radical surgery was the treatment of choice for several decades. However, even at highly-specialized and experienced surgical facilities radical surgery can result in severe damage to the visual apparatus and hypothalamic-pituitary axes, leading to hypothalamic disorders such as obesity. Involvement and/or lesions of posterior hypothalamic areas are associated with severe hypothalamic sequelae. Materials and methods: We compared the grade of pre-surgical hypothalamic involvement, the treatment, degree of resection and grade of surgical hypothalamic lesions between patients recruited in KRANIOPHARYNGEOM 2000 (n = 120; 2001 – 2007) and KRANIOPHARYNGEOM 2007 (n = 106; 2007 – 2012). Results: The grade of initial hypothalamic involvement was similar in patients treated 2001 – 2007 and 2007 – 2012. The intention-to-treat was more radical (p = 0.01) in patients recruited 2001 – 2007 (38%) when compared with patients treated 2007 – 2012 (18%). In patients with pre-surgical involvement of anterior/posterior hypothalamic areas, the rate of hypothalamus-sparing operations resulting in no (further) hypothalamic lesions was higher (p = 0.005) in patients treated 2007 – 2012 (35%) in comparison with the 2001 – 2007 cohort (13%). Event-free-survival rates were similar in both cohorts. Conclusions: A trend towards less radical surgical approaches is observed, which resulted in a reduced rate of severe hypothalamic lesions. Event-free survival was not compromised by this development. Radical surgery is not an appropriate treatment strategy in patients with hypothalamic involvement. Treatment should be confined to experienced multidisciplinary teams.