Endocrine Abstracts

Introduction: The management of Craniopharyngioma is associated with significant long-term morbidity. We retrospectively assessed the endocrine, hypothalamic and neuro-developmental morbidity (at most recent clinic review) in survivors of Craniopharyngioma diagnosed between 1/01/98 and 31/12/09, and currently being managed at our centre.

Methods: We identified 63 patients in our cohort of which 25 were randomly selected for analysis (11 males, 14 females). Eight-four percentage (n=21) underwent definitive debulking surgery (complete resection (CR) in 33% {n=7}, and partial resection (PR) in 66.6% {n=14}), and 68% (n=17) received adjuvant radiotherapy (DXT) at initial presentation. Disease recurrence was seen in 11 (44%) patients: CR: n=4; PR: n=6; DXT: n=7.

Results: Ninety-two percentage (n=23/25) had more than two pituitary hormone deficiencies with GH and TSH deficiencies being the most prevalent (88%; n=22). Twenty patients had ACTH deficiency, whilst 16 had DI. BMI data was available in 16 patients; 50% had BMI >30.0 kg/m², and the mean BMI was 30.49 kg/m². Neuro-developmental data was available in 23 patients; 69.6% (n=16) were either in employment, higher education, or attended mainstream schools, 26.1% (n=6) attended mainstream schools, but had educational needs (4 of which required assistance at school), and 1 patient attended a special needs school. Behavioural issues (mainly related to anger about their condition, excessive weight gain, and subsequent isolation at school) requiring psychology input was observed in 6 patients. Of the 25 patients, 4 were registered blind, 5 had a history of sleep disorder, and 1 had temperature dysregulation.

Conclusion: The majority of our Craniopharyngioma patients diagnosed in the last decade have had partial debulking surgery followed by adjuvant radiotherapy at initial presentation. Endocrine morbidity remains highly prevalent and 50% of our patients were obese at their last clinic review. Neuro-developmental outcomes are however encouraging as 70% presently (current mean age: 13.03 years) do not require cognitive assistance.