ESPE Abstracts (2018) 89 P-P1-195

Long Term Reversibility of Presumed ACTH Deficiency (ACTHd) in Children and Young People (CYP) with Intracranial Germ Cell Tumours (IGCT)

Kyriaki Pieri, Maria Michaelidou, Zaynab Chatoo, Ross Holloway, Antonia Dastamani & Helen A Spoudeas


London Centre for Paediatric Endocrinology, Neuroendocrine Division, Great Ormond Street (GOSH) and University College (UCLH) Hospitals, London, UK


Introduction: ACTHd is life-threatening and difficult to differentiate from ACTH suppression (ACTHs) especially in CYP receiving perioperative corticosteroids. In our experience, this is always the most robust anterior pituitary hormone to brain injury, whilst GH deficiency (GHd) is the first and LH/FSHd and TSHd intermediate in hierarchy. We previously showed HPA axis recovery at 3.08 (2.38–10.33) years after cortiscosteroid therapy for ACTHd in 13.6% of 44 CYP with craniopharyngioma and ACTHd, and showed that intact TSH and post-pubertal LH/FSH axis and pre-dose ACTH>10 ng/L are predictive of recovery.

Aim: To assess the same recovery rates in CYP with IGCT and analyse data by tumour position.

Methods: 46 CYP with IGCT (24M) were identified from local databases and longitudinal records retrospectively reviewed.

Results: At diagnosis, CYP were aged 10.78 (5.13–17.87) years and followed for 7.92 (0.75–24.18) years. Of these 46 CYP, 13 (28.3%) had pineal, 24 (52.2%) suprasellar and 9 (19.6%) bifocal IGCT. All but 14 (30.4%) CYP had ACTHd 32/46 (69.6%). Of these 14 with intact ACTH, 8 (57.1%) had pineal, 4 (28.6%) suprasellar and 2 (14.3%) bifocal IGCT. Of these 14, 8/10 (80%) with data available had GHd, 3/10 (30%) TSHd, 0/10 (0%) LH/FSHd and 1/10 (10%) ADHd at last review. 6/32 (18.8%) with presumed ACTHd discontinued hydrocortisone after 3.79 (0.02–6.16) years with adrenal reserve ‘recovery’ and detectable ACTH 23.05 ng/l (15.9–26.2). Of these, 2/6 (33.3%) had pineal, 3/6 (50%) suprasellar and 1/6 (16.7%) bifocal IGCT. At latest follow-up, 4/6 (66.7%) had GHd, 2/6 (33.3%) TSHd, 1/6 (16.7%) LH/FSHd and 2/6 (33.3%) ADHd. The remaining 26/46 (56.5%) ACTHd CYP continue on hydrocortisone with ACTH<0.7 ng/l (<0.7–25.0), 7.59 (1.33–24.18)years later. Of these, 3/26 (11.5%) had pineal, 17/26 (65.4%) suprasellar and 6/26 (23.1%) bifocal IGCT. Of these 26, 19/21 (90.5%) with data available had GHd, 19/21 (90.5%) TSHd, 16/21 (76.2%) LH/FSHd and 18/21 (85.7%) ADHd. A persisting hydrocortisone requirement was significantly associated with co-existing TSH, LH/FSHd and ADHd (P<0.01). CYP remaining on hydrocortisone showed greater increment in BMISDS than those with intact ACTH (P=0.039).

Conclusion: Interval reassessment of the HPA axis in CYP with suprasellar disease shows recovery in 18.8% of CYP at 4 years follow-up. It is vital to differentiate ACTHs from ACTHd to avoid secondary obesity and overdiagnosis attributable to therapy. A pineal tumour position, a detectable pre-dose ACTH and intact TSH or LH/FSH axis increase the likelihood of intact ACTH.

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