gms | German Medical Science

Objective: Multivariable analyses of risk factors (age at diagnosis, degree of resection, irradiation, growth hormone treatment, gender) and descriptive analyses of overall (OS) and event-free survival (EFS) rates were performed in 117 patients from Germany, Austria and Switzerland, recruited prospectively during 2001 and 2006 and evaluated after 3 yrs of follow-up (KRANIOPHARYNGEOM 2000).

Methods: Body mass index (BMI) and QoL (as measured by PEDQOL) at diagnosis, 12 and 36 mo after diagnosis were analyzed in relation to neuroradiological reference assessment of tumor localization and a score of post surgical hypothalamic damage (anterior, posterior or no hypothalamic lesions).

Results: We observed a 3-yrs-OS of 0.97±0.016 and a 3-yrs-EFS of 0.50±0.05, indicating high recurrence rates after complete resection (CR) (n=47; 3-yrs-EFS: 0.63±0.09) and high progression rates after incomplete resection (IR) (n=66; 3-yrs-EFS: 0.31±0.07). The risk of an event decreased by 80% after CR compared to IR (HR=0.20; p<0.001). Irradiation (XRT) had protective effects on EFS. XRT-patients had an 88% lower risk of progression compared to patients without/before XRT (HR=0.12, p<0.001). Growth hormone (GH) substitution therapy had no impact on 3-yrs-EFS. BMI SDS at diagnosis was similar in patients without and with hypothalamic involvement of anterior or posterior hypothalamic areas. Surgical hypothalamic lesions of posterior hypothalamic areas (as detected in postsurgical imaging) were associated with increases in BMI-SDS during the first 12 mo (median increase +2.2 BMI SD; p<0.01) and 36 mo (+3.2 BMI SD; p<0.01). Postsurgical QoL deteriorated in patients with posterior hypothalamic lesions. Postoperative increases of BMI (>2SD) were associated with lowest QoL.

Conclusions: We conclude that tumor recurrences/progressions are frequent and occur early after initial treatment of childhood craniopharyngioma. GH substitution had no impact on high recurrence/progression rates observed during short-term follow-up. A radical surgical strategy leading to damage of posterior hypothalamic areas is not recommended due to associated severe obesity and impaired QoS. XRT was efficient in preventing recurrences/progressions. Accordingly, in KRANIOPHARYNGEOM 2007 (www.kraniopharyngeom.net) QoL and survival rates after IR in patients with childhood craniopharyngioma (>5 yrs of age at diagnosis) are analyzed after randomization of the time point of XRT (immediate XRT vs. XRT at the time of progression of the residual tumor).