Exp Clin Endocrinol Diabetes 2006; 114 - P21
DOI: 10.1055/s-2006-954714

Prognostic impact of hypothalamic involvement in patients with childhood craniopharyngioma – Results of a cross-sectional study and update on the prospective surveillance study KRANIOPHARYNGEOM 2000

HL Mueller 1, U Gebhardt 1, A Faldum 2, F Pohl 3, C Roth 4, M Warmuth-Metz 5, G Calaminus 6, N Soerensen 7
  • 1Department of Pediatrics, Zentrum fuer Kinder- und Jugendmedizin, Klinikum Oldenburg gGmbH, Germany
  • 2Institute for Medical Biometry, Epidemiology and Informatics (IMBEI), Mainz, Germany
  • 3University Hospital for Radiooncology, University Regensburg, Germany
  • 4University Childrens Hospital, Bonn, Germany
  • 5Department of Neuroradiology, University Wuerzburg, Germany
  • 6Klinik fuer Paediatrische Onkologie, Universitaet Duesseldorf, Germany
  • 7Department of Pediatric Neurosurgery, University Hospital Wuerzburg, Germany, *on behalf of the study commission KRANIOPHARYNGEOM 2000

Whereas the overall survival rate in patients with childhood craniopharyngioma (CR) is high (92%), side effects such as severe obesity due to hypothalamic involvement (HI) have major impact on quality of life (QoL) in survivors of CR. We analyzed 212 patients with CR in regard to HI, growth, obesity (body mass index [BMI] SDS) and QoL (FMH scale, PEDQOL questionnaire). In our cross-sectional study 59% of all patients with CR presented with HI at the time of diagnosis. Severe long-term obesity was correlated with HI and found to be a major risk factor for reduced QoL. Patients with CR and HI presented with higher BMI already at the time of diagnosis. Analyzing anthropometric data collected before diagnosis of CR we found reduced growth rates as early as at age of 12 months. Increases in BMI occurred at age of 5 years, shortly before diagnosis of CR. Analyzing pathogenic mechanisms for the development of severe obesity we found that patients with CR and HI showed reduced physical activity as measured by accelerometric movement analysis. Severe daytime sleepiness due to reduced nocturnal melatonin levels were found in patients with HI of CR. Differences in self-assessment of caloric intake between patients with CR and age-, sex- and BMI-matched controls did not reach statistical significance. Aware of possible bias due to retrospective analysis in our cross-sectional study (n=290) we initiated a prospective multicenter study in 2001. Between September 2001 and December 2005 we were able to prospectively analyze 98 patients with CR recruited in KRANIOPHARYNGEOM 2000. We conclude that HI has major impact on BMI, long-term QoL and functional capacity in patients with CR. A major aim of the ongoing study KRANIOPHARYNGEOM 2000 is to analyze the prognostic relevance of therapeutic strategies such as the degree of surgical resection, irradiation and rehabilitative efforts on outcome and QoL especially in patients with HI.

This work was supported by the Deutsche Kinderkrebsstiftung, Bonn, Germany