Analyses of Treatment Variables for Patients with Childhood Craniopharyngioma - Results of the Multicenter Prospective Trial KRANIOPHARYNGEOM 2000 after Three Years of Follow-Up

作者：Mueller Hermann L; Gebhardt Ursel; Schroeder Sabine; Pohl Fabian; Kortmann Rolf Dieter; Faldum Andreas; Zwiener Isabella; Warmuth Metz Monika; Pietsch Torsten; Calaminus Gabriele; Kolb Reinhard; Wiegand Christoph; Soerensen Niels

来源：Hormone Research in Paediatrics, 2010, 73(3): 175-180.

DOI：10.1159/000284358

• Childhood craniopharyngioma
• Growth hormone
• Recurrence
• Survival
• Hypothalamus

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.

• 出版日期2010
• 单位河北医科大学