Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2016) 45 OC1.1 | DOI: 10.1530/endoabs.45.OC1.1

BSPED2016 Oral Communications Oral Communications 1- CME (2 abstracts)

Pan hypopituitarism – Is it secondary to brain tumour and its treatment?

Ved Bhushan Arya & Helen Spoudeas

Great Ormond Street Hospital, London, UK.

A 10-year old boy presented with 12-months history of headache, vomiting, declining school performance and change in behavior. There was no visual disturbance. Neurological examination was normal. Weight and height were between 50th−75th centile. CT head showed a large, partly calcified mass in the sellar region with acute hydrocephalus. Dexamethasone was immediately commenced and care was transferred to the neurosurgical centre.

MRI brain confirmed partly cystic, partly solid intra-and supra-sellar mass with displacement of anterior visual pathway and obstructive hydrocephalus. Pre-operative endocrine investigations showed normal prolactin, normal free T4 with low TSH, normal IGF1/IGFBP3 and normal serum sodium and osmolality. Serum cortisol was low (34 nmol/l), however this was measured post-commencement of dexamethasone.

Initial surgical management involved endoscopic cyst-aspiration and Ommaya reservoir insertion. Dexamethasone was stopped post-surgery and early morning cortisol after 48 hours was satisfactory (364 nmol/l). Subsequently tumor excision was undertaken (histology: adamantinomatous craniopharyngioma). Post-surgery, triphasic response (diabetes insipidus - syndrome of inappropriate ADH secretion - diabetes insipidus) was observed. Management involved intense monitoring of fluid balance, serum and urine electrolytes/osmolality and stat DDAVP doses until permanent diabetes insipidus was clearly established. Perioperative hydrocortisone cover was provided. Measurement of serum cortisol (pre-hydrocortisone dose) at 7 am, 8 am and 9 am (37, 41 and 41 nmol/l) indicated ACTH deficiency. Low fT4 (5.4 pmol/l) and low TSH (0.7 mU/l) post-operatively suggested TSH deficiency.

At discharge, the proband was on DDAVP, Levothyroxine and Hydrocortisone. Post-discharge, investigation with combined pituitary function testing (Growth Hormone [GH] stimulation, LHRH and TRH testing) showed absent GH, LH/FSH and TSH response, confirming GH deficiency, gonadotropin and TSH deficiency. GH treatment was commenced. In view of near-complete tumor resection, multi-disciplinary supra-regional discussion forum decided against radiotherapy treatment. Recurrence of craniopharyngioma was noticed 1-year after surgery, for which a second resection was undertaken after multi-disciplinary discussion.

Conclusions: Pituitary reserve should be assessed, if possible, before surgery to ascertain whether the deficit is secondary to tumor or its treatment. Anticipation of triphasic response (DI-SIADH-DI) and appropriate management is critical. Recurrence rate of craniopharyngioma is high without adjuvant radiotherapy. The difficult decisions regarding optimal treatment strategy are best taken in a supra-regional/national forum.

Volume 45

44th Meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

Browse other volumes

Article tools

My recent searches

No recent searches.