Options Tablets - such as Metyrapone Radiotherapy to the pituitary gland. An operation to remove both adrenal glands.
Options Not easily controlled tablets Tablets would be necessary several years after radiotherapy Recommend bilateral adrenalectomy– refer to surgeon Miss A and family agreed.
Treatment 23/12/1998 Bilateral adrenalectomy - Hydrocortisone 10mg,5mg,5mg Fludrocortisone 100mcg o.d. Pigmentation knuckles and palmar creases Close watch on ACTH TFTs normal on Thyroxine Desmopressin still for Diabetes Insipidus Gonadotrophin deficiency
February 1999 Day curve – levels cortisol good mean 392 nmol/l No features Cushing’s Suppressed Renin levels (Fludrocortisone) ACTH 602ng/l Oestrogen replacement (Mercilon) ?Nelson’s syndrome
Nelson’s Syndrome Rapid enlargement of a pituitary adenoma following bilateral adrenalectomy Lack of negative feedback from cortisol Mass effects Increased production ACTH Increased production melanocyte stimulating hormone. Muscle weakness Hyperpigmentation
June 1999 Progressive darkening skin Increase Hydrocortisone 10mg/10mg/5mg Repeat Hydrocortisone day curve Repeat ACTH levels
September 1999 Blood Cortisol levels and UFC at day curve top end acceptable level mean bloods 559nmol/L UFC 552nmol/24hrs ACTH lower 181ng/L (increased suppression corticotroph adenoma) Weight gain Stretch marks Balancing act MRI
March 2000 DNA MRI appointment October 1999 February 2000 MRI reported as: Macroadenoma on left pituitary fossa extending into cavernous sinus.Not visible on previous MRIs or at surgery. Increasing pigmentation Pituitary radiotherapy –June 2000
September 2000 Pigmentation less Continues Hydrocortisone 10mg/10mg/5mg ACTH still elevated 9000ng/L pre morning Hydrocortisone 640ng/L 2 hours after Weight gain but risk of enhancing growth adenoma if dose Hydrocortisone reduced.
Follow Up 2003-2005 Continued to be stable and no changes in pigmentation MRI July 2003 – adenoma stable ACTH level lower in 2003 Gynae problems GH deficient but declined treatment.
2006 Well No changes in pigmentation ACTH before and after Hydrocortisone Higher than previously (post 808ng/l) Hydrocortisone increased Monitor
2007 Increased pigmentation ACTH pre and 2 hours post Hydrocortisone Post 4,760nmols/l MRI pituitary Significant Lt lateral extension passing through cavernous sinus Significant enlargement compared to previous films mostly laterally but now filling fossa.
Treatment MDT discussion Further de-bulking surgery –but unlikely to be completely resectable as wrapped around carotid and ocular motor nerves Possible gamma knife therapy Cabergoline –no effect
Trans-sphenoidal Hypophysectomy December 2007 Post op MRI – encouraging but many ‘scars of battle’ Plan further MRI June ACTH 2 hour post hydrocortisone 133ng/L June MRI – Good clearance but possible small amount residual tissue. Continue monitoring ACTH and MRI
June 2008 ACTH Pre and post Hydrocortisone levels Pre >1250ng/L Post 423ng/L