High rates of early relapses after complete resection and early tumor progressions after incomplete resection of childhood craniopharyngioma – Update after three years of prospective evaluation in KRANIOPHARYNGEOM 2000 and study design of KRANIOPHARYNGEOM 2007

In our multicenter cross-sectional study HIT-ENDO we collected data on therapy and outcome of 306 patients with childhood craniopharyngioma (CP). The survival rates were 94±4% in irradiated and 93±5% in non-irradiated patients. The German multicenter prospective study KRANIOPHARYNGEOM 2000 (www.kraniopharyngeom.de) was initiated in order to collect data on incidence and time course of relapses after complete surgery and tumor progressions after incomplete resection. Furthermore, the impact of irradiation (XRT) on relapse and recurrence rates was analyzed. Since 2001 ninety-six patients with newly diagnosed CP were recruited. With a high degree of completeness (80–90%) data on neurosurgery, neuroradiology and XRT could be collected prospectively. Complete resection was achieved in 43%, subtotal resection in 45%. XRT was performed in 22 of 96 CP patients; in 18% immediately after subtotal resection, in 53% after progression of residual tumour and in 14% after (second surgery of) relapse. Data on XRT modalities were evaluable in 17 of 22 patients. XRT was performed at a median age of 11 years (4–18 y) and after a mean interval of 10 months after first diagnosis. All patients got a 3-dimensional CT-planning of XRT. The mean total dose was 52.5 Gray. An interim evaluation on event-free survival rates (EFS) after three years of follow-up showed a high rate of early events (EFS: 0.22±0.06) in terms of tumour progression after subtotal resection (n=48) and relapses (EFS: 0.60±0.10) after complete resection (n=37) during the first three years of follow-up. A high rate of early events (EFS: 0.57±0.15) was also found for patients after XRT (2 zystic progressions, 4 progressions of solid tumour parts after XRT). We conclude that progression after subtotal resection and relapse after complete resection of CP are frequent and early events even in irradiated patients during the first three years after diagnosis. Regular monitoring of cerebral imaging and clinical status is recommended in follow-up of patients with CP. There is controversial discussion on the adequate time point of irradiation after incomplete resection. Accordingly, the multicenter prospective study KRANIOPHARYNGEOM 2007 will focus on this issue. Patients at age ≥5 years at diagnosis will be randomized after incomplete resection for the time point of irradiation (immediate XRT after surgery versus XRT at progression of residual tumour). Endpoints of the study will be quality of life (PEDQOL domaine: physical function), progression-free survival and overall survival). The time point of evaluation will be 3 years after randomisation.

Supported by Deutsche Kinderkrebsstiftung, Bonn, Germany