Craniopharyngioma in childhood: our evidence-based approach to management

In 1996 we published our results for treatment of childhood craniopharyngioma. That study did not only reveal that there was a significant morbidity associated with our then policy of attempted radical removal followed by post-operative radiotherapy in those cases with residual disease, but also that risk factors for poor outcome could be identified based on the clinical and radiological findings at presentation. As result of that study, we redefined the role of radical surgery in the treatment of craniopharyngioma and developed a new treatment strategy in an attempt to improve the quality of outcome without compromising tumour control. Our aims in this paper were to compare the results of our current treatment strategy with that reported in the 1996 paper to assess whether we have achieved this goal.

A detailed assessment of the treatment pathway and outcome was undertaken for children treated for craniopharyngioma in our unit from 1996 to 2004. This included a morbidity score based on visual, motor, cognitive, hypothalamic and endocrinological data obtained from our neuro-oncology database and review of clinical records. Where possible we have attempted to record data in the same manner as for our previous study allowing for meaningful comparison.

Forty-eight children with craniopharyngioma presented in the study period. On the basis of clinical presentation and radiological findings, 25 were deemed suitable for attempted radical surgery and 23 were treated with various subtotal surgical procedures. Radiotherapy was used in patients over the age of 5 years where residual tumour was present or progressed after the initial surgical intervention(s). Morbidity scores, particularly in relation to visual and cognitive outcome, are improved and there was no surgical mortality in the current series.

A treatment paradigm for childhood craniopharyngioma is presented which improves the quality of outcome without compromising tumour control.