Endocrine Abstracts

Background: Craniopharyngiomas are rare tumours with an annual incidence of 0.5–2/100 000. Though benign they still represent a management challenge because of morbidity that includes pituitary hormone deficiency, visual impairment, adipsia and morbid obesity. We retrospectively assessed the outcomes of craniopharyngioma patients in our centre over a 10-year period.

Methods: All children (n=16) diagnosed with craniopharyngioma and treated in this centre from 1996 to 2011 were identified. Clinical data from presentation to most recent assessment were collected. MRI scans were reviewed with a neuroradiologist. Height, weight and BMI were expressed as standard deviation scores (SDS).

Results: Mean age at diagnosis was 11.2 years (range 3.3–16.1) and they were followed up for a mean of 8.6 years (3.4–16.2). Tumour dimensions ranged from between 12 and 15 mm to between 35 and 41 mm. 56% had radiotherapy after surgery. 75% had biochemical evidence of pituitary dysfunction at presentation. Postoperatively all had 3 or more endocrinopathies. All were GH/ACTH deficient and all but 1 had cranial DI. Transient adipsia was present in 25% postoperatively. Median height SDS was −1.89 (−2.50 to 0.06), increasing on GH replacement to +0.09 (−1.78 to 1.54). 80% reached a final height greater than their mid-parental target. Median BMI SDS at presentation was 1.23 (−1.55 to 3.43), increasing to 2.10 (−0.14 to 3.72) at 2 years and 2.24 (−0.06 to 4.75) at last follow up. 2 patients developed type 2 DM. At presentation 44% had visual field defects, increasing to 63% postoperatively.

Conclusion: The outcome in patients with craniopharyngioma in our unit is similar to other larger UK centres. Endocrinopathies are common at presentation and universal postoperatively but no patient had adipsic DI in the long term. Despite short stature at presentation all have a final height within normal limits. BMI is high at presentation and rises postoperatively but may plateau in the long term.