Analyses of treatment variables for patients with childhood craniopharyngioma--results of the multicenter prospective trial KRANIOPHARYNGEOM 2000 after three years of follow-up.

Abstract

BACKGROUND:

Controversies surround various treatment variables for patients with childhood craniopharyngioma such as growth hormone (GH) replacement, which some believe can exacerbate recurrence/progression. We prospectively assessed the risk of tumor recurrence/progression in survivors of childhood craniopharyngioma.

METHODS:

Multivariable analyses of risk factors (age at diagnosis, degree of resection, irradiation, GH treatment and gender) and descriptive analyses of overall survival (OS) and event-free survival (EFS) rates were performed in 117 patients, recruited prospectively and evaluated after 3 years of follow-up in the German, Austrian and Swiss multicenter trial KRANIOPHARYNGEOM 2000.

RESULTS:

We observed a 3-year OS of 0.97 and a 3-year EFS of 0.46, indicating high recurrence rates after complete resection (CR) (n = 47; 3-year-EFS: 0.64) and high progression rates after incomplete resection (IR) (n = 64; 3-year EFS: 0.31). The risk of an event decreased by 80% after CR compared to IR (hazard ratio = 0.20; p < 0.001). Irradiation had protective effects on EFS: irradiated patients had an 88% lower risk of recurrence/progression compared to patients without/before irradiation (hazard ratio = 0.12; p < 0.001). GH treatment had no impact on 3-year EFS rates.

CONCLUSIONS:

Tumor recurrences/progressions are frequent and occur early after initial treatment of childhood craniopharyngioma. A radical resection preserving the integrity of hypothalamic structures appears optimal at original diagnosis. Irradiation was efficient in preventing recurrences/progressions. GH treatment had no impact on the low 3-year EFS observed in our study. However, further conclusions on the influence of GH on recurrence rates have to be refined to long-term follow-up studies of patients with childhood craniopharyngioma.

• Authors: Hermann L Müller, Ursel Gebhardt, Sabine Schröder, Fabian Pohl, Rolf-Dieter Kortmann, Andreas Faldum, Isabella Zwiener, Monika Warmuth-Metz, Torsten Pietsch, Gabriele Calaminus, Reinhard Kolb, Christoph Wiegand, Niels Sörensen, study committee of KRANIOPHARYNGEOM 2000/2007
• Journal: Hormone research in paediatrics (Horm Res Paediatr) Vol. 73 Issue 3 Pg. 175-80 ( 2010) ISSN: 1663-2826 [Electronic] Switzerland
• PMID: 20197669 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
• Copyright: Copyright 2010 S. Karger AG, Basel.

Chemical References

• Human Growth Hormone

Topics

• Adolescent
• Child
• Child, Preschool
• Craniopharyngioma (drug therapy, radiotherapy, surgery, therapy)
• Disease Progression
• Disease-Free Survival
• Female
• Follow-Up Studies
• Human Growth Hormone (therapeutic use)
• Humans
• Infant
• Male
• Multivariate Analysis
• Neoplasm Recurrence, Local (prevention & control)
• Pituitary Neoplasms (drug therapy, radiotherapy, surgery, therapy)
• Prospective Studies
• Risk Factors
• Treatment Outcome