Analyses of treatment variables for patients with childhood craniopharyngioma--results of the multicenter prospective trial KRANIOPHARYNGEOM 2000 after three years of follow-up.
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.
Müller
Hermann L
HL
Department of Pediatrics, Zentrum für Kinder- und Jugendmedizin, Klinikum Oldenburg gGmbH, Oldenburg, Germany. mueller.hermann@klinikum-oldenburg.de
Gebhardt
Ursel
U
Schröder
Sabine
S
Pohl
Fabian
F
Kortmann
Rolf-Dieter
RD
Faldum
Andreas
A
Zwiener
Isabella
I
Warmuth-Metz
Monika
M
Pietsch
Torsten
T
Calaminus
Gabriele
G
Kolb
Reinhard
R
Wiegand
Christoph
C
Sörensen
Niels
N
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003397:Craniopharyngioma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D019382:Human Growth Hormone / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008297:Male; D015999:Multivariate Analysis; D009364:Neoplasm Recurrence, Local / Q000517:prevention & control; D010911:Pituitary Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D011446:Prospective Studies; D012307:Risk Factors; D016896:Treatment Outcome
D019382:Human Growth Hormone