We report a 61year old female who presented with severe Cushing's disease 13 years after diagnosed as non-functioning adenoma. First presented in 1992 with visual field defect. MRI showed macroadenoma, suprasellar, and lateral extension. Gonadotrophin and GH were low. At this time there was no clinical suspicion of Cushing's syndrome. Treated with transphenoidal pituitary surgery resulting in improved vision. Immunohistochemistry showed 50% cells positive for ACTH and negative for other hormones. She underwent radiotherapy for the residual tumour. April 200 complained of blurred vision and MRI showed enlarging pituitary lesion. Hormone profile showed hypothyroid, normal adrenal axis. Repeat pituitary surgery failed to improve vision or reduced the size of tumour. Referred for Gamma knife radiosurgery but was advised debulking operation which patient refused. Octreotide LAR was tried with no improvement in tumour size or symptoms. Feb. 2002, diagnosed as type 2 diabetes and hypertension. Defaulted from clinic.
June 2003, referred for deterioration in her condition with weakness and tiredness. Diagnosed florid Cushing with biochemical confirmation. MRI confirms aggressive pituitary tumour.
Treated with Ketaconazole to prepare for urgent bilateral Adrenalectomy. Her clinical condition deteriorated and could not take oral medications. Etomidate intravenous infusion 60 milligrams daily started. Plasma cortisol level was reduced from 1677ninimol per litre to 38l. Patient continued to deteriorate with loss of consciousness. CT head showed hydrocephalus requiring urgent VP shunt with no clinical improvement. While on Etomidate she had respiratory arrest and was successfully resuscitated but died later.
This case shows that recurrence of Cushing's syndrome in a non-functioning pituitary adenoma could be severe with poor prognosis and the Etomidate is a safe and effective treament in those patients who are unable to take oral medication.
22 - 24 Mar 2004
British Endocrine Societies