Introduction: The treatment of craniopharyngiomas remains controversial in the absence of class I and class II data. Despite strong opinions advocating aggressive versus conservative approaches little longterm outcome data exist. Conventional wisdom suspects drainage of craniopharyngioma cysts into the ventricle to cause aseptic meningitis/ventriculitis. We studied this approach systematically in a large group of patients followed for more than 15 years.
Methods: Patients with mixed solid/cystic craniopharyngiomas were treated consistently with stereotactic cyst-ventricular shunt followed immediately by fractionated limited field radiotherapy ( 54 Gy). Multivariate analysis for prognostic variables was performed including cyst volume, visual outcome,endocrinological outcome, tumor control, overall survival and progression free survival. Visual and endocrinological outcome was assessed by an independent outside ophthalmologist/endocrinologist.
Results: 59 patients were included with a mean age of 12.6+4.8 years and a median follow-up of 17.5+4.3 years. Tumor control was achieved in 93.2 % of patients and cyst volume showed a median reduction of 87+12 %. Visual outcome showed improvement in 59 %, stable disease in 39 % and deterioration in 2%. Endocrinological deterioration requiring new substitution occurred in 4 % with 86% stable endocrinological findings and 10 % improvement of the hypothalamic-pituitary axis impairment compared to preoperative findings. Overall survival and progression free survival could not be calculated because of lack of events in the Kaplan-Meyer plots. No cases of aseptic meningitis/ventriculitis were observed despite drainage of up to 80 cc of cyst fluid into the CSF.
Conclusions: This is the largest series of craniopharyngiomas treated with cyst-ventricular shunting and radiotherapy with the longest follow-up.
It shows that aseptic meningitis is vastly overestimated and a never event in this series. Longterm outcome of this approach is significantly superior to aggressive resection with concomitantly lower morbidity/mortality.
Patient Care: The study shows the value of a conservative stereotactic approach of internal cyst drainage and radiotherapy as a longterm strategy to preserve function and quality of life in patients. It invalidates the assumption that intraventricular drainage causes aseptic meningitis/ventriculitis.
Learning Objectives: By the conclusion of this session participants should be able to: 1) Understand the concept of cyst-ventricular shunting in craniopharyngiomas. 2) Assess the role of conservative versus aggressive approaches to craniopharyngioma treatment. 3) Develop a treatment algorithm to assure longterm quality of life in craniopharyngioma patients.