LONG-TERM RESULTS OF TREATMENT FOR CRANIOPHARYNGIOMA IN CHILDREN

Results of the treatment of 27 children with craniopharyngioma are rep orted. A subfrontal pterional approach was used in 55.6% of cases, a t ranssphenoidal and a transcallosal or transcortical approach in 25.9% and 18.5% of cases, respectively. Radical removal was the goal of surg ery and was achieved in 70.8% of cases treated as primary surgery. The operative mortality was 3.7% and was due to hypothalamic failure. Mos t (81.4%) patients were followed up, for a mean of 7 years. Patients w ere evaluated according to a functional evaluation scale and outcome c ategories proposed by us. The scale takes account of tumor (recurrence s and their eventual evolution); visual functions; endocrine functions (mainly hypothalamic), attainment of endocrine balance and drug regim en; headache; and psychosocial function. Recurrences were observed in 17.6% of patients treated with radical surgery and in 42.8% of those t reated with limited surgery plus radiotherapy. A progressive ameliorat ion of visual, endocrine and neuropsychosocial functions from the inte rvention to follow-up was observed in the majority of patients. Comple te tumor excision was associated in 85% of cases with a low score on t he functional scale, reflecting a high functional performance. Adequat e substitution therapy maintained endocrine balance in 81% of patients . Since the intervention a progressive decrease in the number and dosa ges of medications has been observed. The majority of patients were ag ain able to lead a normal social life. Small stature, obesity, headach e, and emotional and sexual disturbances were frequent cause of long-t erm disability even despite adequate drug regimens. The functional eva luation scale we propose is a simple and effective tool that can be ea sily used during routine evaluation of patients with craniopharyngioma .