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Endocrine Nurse’s Conference

Endocrine Nurse’s Conference. Cushing’s Disease. Veronica Kieffer MA BSc (Hons) RGN Endocrine Nurse Specialist Leicester Royal Infirmary, Leicester. History. October 1997 Miss A 16 year old girl Moon face Hirsutism Central obesity Purple striae Proximal myopathy Primary amenorrhoea.

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Endocrine Nurse’s Conference

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  1. Endocrine Nurse’s Conference Cushing’s Disease Veronica Kieffer MA BSc (Hons) RGN Endocrine Nurse Specialist Leicester Royal Infirmary, Leicester

  2. History October 1997 Miss A • 16 year old girl • Moon face • Hirsutism • Central obesity • Purple striae • Proximal myopathy • Primary amenorrhoea

  3. Investigations • Urea and Electrolytes (U&E) • Liver Function Test (LFT) • Thyroid function tests (TFTs) • LH/FSH • Oestrogen • Prolactin • Growth hormone • Cortisol • Adrenocorticotrophic hormone (ACTH) • 24 hour Urinary Free Cortisol (UFC)

  4. Results • LFTs, U&Es, TFTs –all normal • LH 0.9iu/L • FSH 2.8iu/L • Prolactin 120iu/L ( 50-400 iu/L) • Oestradiol <50pmol/L • Cortisol 568nmol/L • UFC 257 nmol/24hours (28-221nmol/24hrs) • ACTH 60ng/L (0-50ng/L)

  5. Further Investigations • Circadian rhythm cortisol • Midnight cortisol • Low dose Dexamethasone suppression test • CRH test • MRI Pituitary gland • CT Adrenal glands • Petrosal Sinus Catheter

  6. RESULTS Circadian rhythm • 0930 --- 556nmol/L • 1200 --- 663nmol/L • 1700 --- 635nmol/L • Mean --- 618nmol/L • Midnight cortisol --- 490nmol/L Low Dose Dexamethasone Test Dex 2+0 --- 568nmol/L Dex 2+48--- 246nmol/L

  7. Results CRH Test No change in cortisol levels so not useful in diagnosing cause MRI Pituitary ?Right Pituitary microadenoma CT Adrenals No lesion found

  8. Petrosal Sinus Catheter

  9. Treatment • Metyrapone 19/11/1997 Cortisol levels on Metyrapone 750mg stat • 0900 – 554nmol/l • 1200 – 79 nmol/l • 1700 – 107nmol/l 18/12/1997 Cortisol levels on Metyrapone 250mg t.d.s • 0900 – 120nmol/l • 1200 – 135nmol/l • 1430 – 89nmol/l • 1700 – 109nmol/l • Mean 113nmol/l

  10. Treatment 9th January 1998 0900 cortisol – 656 nmol/l 19/1/1998 Cortisol levels on Metyrapone 250mg nocte • 0900 – 514nmol/l • 1200 – 674nmol/l • 1430 – 620nmol/l • 1700 – 493nmol/l • Mean -- 575nmol/l • Block and Replace • Metyrapone 250mg t.d.s / Hydrocortisone10mg o.d.

  11. Treatments for Cushing’s Disease. • Trans-sphenoidal removal of tumour. • Remission in 75-80% cases • External pituitary radiation • Slow acting. Effective in 50-60% cases. • Medical Therapy to reduce ACTH (Bromocriptine) • Rarely effective • Bilateral Adrenalectomy • Effective last resort

  12. Surgery • Trans-sphenoidal hypophysectomy 10/03/1998 • 18/03 - 0900 cortisol --- 768nmol/l • 19/03 - 0900 cortisol --- 992nmol/l • Trans-sphenoidal hypophysectomy 17/04/1998 • 22/04 - 0900 cortisol --- 738nmol/l • 23/04- 0900 cortisol --- 624nmol/l • 25/04 -0900 cortisol --- 596nmol/l • Pan hypopituitarism with diabetes insipidus • Restart ‘Block and Replace’

  13. Block and Replace 16/11/1998 Metyrapone 250mg t.d.s/ Hydrocortisone 10mg o.d. • 0930 --- 555nmol/l • 1200 --- 273nmol/l • 1430 --- 103nmol/l • 1700 --- 101nmol/l • Mean 258nmol/l • 24 hour UFC 63nmol/24hours

  14. Options • Tablets - such as Metyrapone • Radiotherapy to the pituitary gland. • An operation to remove both adrenal glands.

  15. Options • Not easily controlled tablets • Tablets would be necessary several years after radiotherapy • Recommend bilateral adrenalectomy– refer to surgeon • Miss A and family agreed.

  16. 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

  17. 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

  18. 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

  19. June 1999 • Progressive darkening skin • Increase Hydrocortisone 10mg/10mg/5mg • Repeat Hydrocortisone day curve • Repeat ACTH levels

  20. 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

  21. 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

  22. 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.

  23. ACTH levels

  24. 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.

  25. 2006 • Well • No changes in pigmentation • ACTH before and after Hydrocortisone • Higher than previously (post 808ng/l) • Hydrocortisone increased • Monitor

  26. 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.

  27. 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

  28. Trans-sphenoidal HypophysectomyDecember 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

  29. June 2008 • ACTH Pre and post Hydrocortisone levels • Pre >1250ng/L • Post 423ng/L

  30. Conclusion • Cushing’s syndrome • 2 transsphenoidal hypophysectomies • Bilateral adrenalectomy • Nelson’s syndrome • Radiotherapy • 3rd transsphenoidal hypophysectomy • ? Nelson’s recurring

  31. The Future? • Watchful wait • Gamma knife therapy • No further radiotherapy after that • Repeated de-bulking surgery • Careful monitoring

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