Childhood craniopharyngioma - changes of treatment strategies in the trials KRANIOPHARYNGEOM 2000/2007

Klin Padiatr. 2014 May;226(3):161-8. doi: 10.1055/s-0034-1368785. Epub 2014 May 12.

Abstract

Background: Prognosis in childhood cranio-pharyngioma, is frequently impaired due to sequelae. Radical surgery was the treatment of choice for decades. Even at experienced facilities radical surgery can result in hypothalamic disorders such as severe obesity.

Objective: We analyzed, whether treatment strategies for childhood craniopharyngioma patients recruited in GPOH studies have changed during the last 12 years.

Materials and methods: We compared the grade of pre-surgical hypothalamic involvement, 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 realized treatment 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 was accompanied by a reduced rate of severe hypothalamic lesions. Radical surgery is not an appropriate treatment strategy in patients with hypothalamic involvement. Despite previous recommendations to centralize treatment at specialized centers, a trend towards further decentralization was seen.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Austria
  • Belgium
  • Body Mass Index
  • Child
  • Child, Preschool
  • Clinical Trials as Topic
  • Cohort Studies
  • Craniopharyngioma / mortality
  • Craniopharyngioma / pathology*
  • Craniopharyngioma / surgery*
  • Disease-Free Survival
  • Female
  • Germany
  • Humans
  • Hypophysectomy / methods*
  • Hypophysectomy / trends*
  • Hypothalamic Diseases / etiology
  • Hypothalamic Diseases / mortality
  • Hypothalamus / pathology
  • Hypothalamus / surgery*
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Neoplasm Grading
  • Neoplasm Invasiveness / pathology
  • Obesity / etiology
  • Obesity / mortality
  • Pituitary Neoplasms / mortality
  • Pituitary Neoplasms / pathology*
  • Pituitary Neoplasms / surgery*
  • Postoperative Complications / etiology
  • Postoperative Complications / mortality
  • Prognosis
  • Quality of Life
  • Switzerland