SFENCC2021 Abstracts Highlighted Cases (71 abstracts)
Recurrence of cushing’s disease after several years of remission
Department of Endocrinology, Salford Royal Hospital, Manchester, United Kingdom
68 Y M Has Background of Hypertension and Asthma. Presented with progressive visual deterioration for 12 months in 2012. VF testing confirmed Bitemporal Hemianopia. He has symptoms suggestive of cortisol excess. Therefore, MRI pituitary and pituitary hormone profile was done. MRI showed pituitary macroadenoma A Pituitary Hormone profile in 2012 showed cortisol excess at value of 755 nmol/l and ACTH 156 ng/l(0–46). IGF-1 17.2 nmol/l which is normal. LDDST was done and cortisol was not suppressed level was 453 nmol/l. At this stage the patient was diagnosed with Cushing’s disease secondary to ACTH producing pituitary macroadenoma with partial hypopituitarism. Therefore, he started on Metyrapone 250 mg BD which titrated according to cortisol level, thyroxine 50 mcg and Tostran Gel 2%. In November 2012 he underwent Transsphenoidal surgical debulking of invasive pituitary macroadenoma. Histology showed densely granulated corticotroph adenoma with raised Ki 67 index in excess of 10% raising the possibility of tumor recurrence and aggressive tumor. Immediate post operative period was uneventful and the patient has had some visual field improvement. He remained off Metyrapone post OP and early morning cortisol level remained significantly elevated (1160 nmol/l) in keeping with tumor residual which was not surprising. Post OP MRI showed good tumor debulking although there was a small part of the suprasellar component extending into the floor of the third ventricle. Hence, Metyrapone 500 mg TDS was restarted and In view of postoperative persistent active cushing and high Ki67 index he was referred for radiotherapy. He completed radiotherapy in 2014 and achieved biochemical remission, confirmed by Post radiotherapy GST and ACTH level, hence metyrapone was stopped. He remains well between 2014 and 2020 with stable tumor residual and biochemistry. Currently, admitted with symptoms and signs of cortisol excess, which confirmed recurrence of Cushing’s disease with very high values of ACTH 477 ng/l, cortisol of 1061 nmol/l and LDDST cortisol value of 894 nmol/l. The MRI pituitary showed recurrence of pituitary macroadenoma.
Discussion: 1. Recurrence of Cushing’s disease after several years of remission 2. Management options are quite limited as no clear surgical Target, specially on background history of radiotherapy 3. Discussion whether to continue with medical treatment or bilateral adrenalectomy