Background: Craniopharyngiomas are rare suprasellar tumours with good survival but high endocrine morbidity. The commonest endocrinopathy is GHd which can precede diagnosis. Later obesity (BMI >+2SDS) may be treatment or tumour related.
Objective and hypotheses: To evaluate endocrinopathy, height, weight and BMI outcomes after a conservative surgical treatment strategy with pre-treatment pituitary provocation tests for children diagnosed between 2009 and 2014.
Method: Retrospective longitudinal review of 21 patient electronic case notes (14M, 7F) diagnosed at median age of 7.8 (range 1.917.18) years and followed up for 3.15 (0.525.37) years. We calculated SDS for Mid-parental height (MPH), height, weight and BMI and noted endocrinopathy at diagnosis and latest follow up. We used the Paris classification to grade the degree (02) of hypothalamic involvement and analysed paired data with non-parametric statistics.
Results: The current prevalence of endocrinopathies follows the hierarchy of GHd (100%), TSHd (80.9%), Gnd (66.7%), ACTHd (66.7%), DI (47.6%) and obesity (28.5%). At diagnosis, 9 (42.9%) patients had pre-existing GHd (peak 1.40 (<0.13.30)) and Grade 0 (n=2), 1 (n=1) or 2(n=6) tumour. The remaining 12 (57.1%) patients with Grade 1 (n=2) or 2 (n=10) tumours developed GHd 0.15 (0.011.81) years after treatment (peak 1.00 (0.26.60)). Overall increments in height, weight and BMI SDS occurred with GH replacement. This was significant for weight (P=0.023). Six patients became obese (1 Grade 1, 5 Grade 2), five of whom did not have GHd at diagnosis. Children with GHd at presentation remain significantly shorter than those without (P=0.031) and are also below MPHSDS (P=0.043). During GH therapy 5 (20%) patients with Grade 2 tumours required delayed radiation to residual for relapse.
Conclusion: Prospective dynamic GH assessment identifies a highly susceptible cohort for GHd and obesity, especially those with Grade 2 tumours. Prompt diagnosis and GH treatment in conjunction with a conservative treatment strategy, improves auxology outcomes with likely benefits on long term health.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology