Endocrine Abstracts

Despite high survival rates (92%) in patients with childhood craniopharyngioma (CP), quality of life (QoL) is frequently impaired due to sequelae such as severe obesity resulting from hypothalamic involvement of CP. Based on the results of the multicenter prospective study KRANIOPHARYNGEOM 2000 radical surgery is no appropriate treatment strategy in patients with hypothalamic involvement. Furthermore, tumour progression/relapses are frequent early events in CP patients. The analysis of event-free survival-rates (EFS) in 117 prospectively evaluated patients with CP showed a high rate of early events in terms of tumour progression after incomplete resection (EFS: 0.31±0.07) and relapses after complete resection (EFS: 0.63±0.09) during the first 3 years of follow-up.

Accordingly, in KRANIOPHARYNGEOM 2007 QoL, and survival rates in CP pts (>5 years at diagnosis) are analyzed after randomization of the time point of irradiation (XRT) after incomplete resection (immediate XRT vs XRT at progression of residual tumour). Up to now (November 2011) 93 pts with CP were recruited (53 pts in the randomization arm; 40 pts in the surveillance arm; 4 pts in the process of review of imaging). 15 of 53 pts were randomized. 34 pts could not be randomized due to parental decision (13 pts), late schedule (12 pts) and due to decision of the physician (5 pts).

In conclusion, KRANIOPHARYNGEOM 2007 represents the first randomized trial in CP and the first study in pediatric neurooncology analyzing QoL as an endpoint. Aim of the study is to analyze the appropriate time point of XRT in order to improve QoL in patients with hypothalamic involvement. The recruiting compliance is high. However, the randomization compliance has to be improved in order to reach cohort sizes necessary for reliable statistical analysis and to answer the questions assessed by the randomized trial KRANIOPHARYNGEOM 2007.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This work was supported, however funding details unavailable.