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Surgery for Cushing’s Disease

  • Brooke SwearingenEmail author
Chapter
Part of the Contemporary Endocrinology book series (COE)

Abstract

Transsphenoidal surgery is considered a first-line therapy for Cushing’s disease in children. Preoperative evaluation includes appropriate biochemical testing, MRI imaging, and inferior petrosal sinus sampling, to confirm a pituitary source. Remission rates between 50% and 95% have been reported in various series, depending on the size and invasiveness of the tumor, with acceptably low surgical morbidity. Recurrence remains an issue despite initial indications of remission, and adjunctive therapy, including radiosurgery, medical treatment, and adrenalectomy, is sometimes required.

Keywords

Pediatric Cushing’s disease Transsphenoidal surgery Inferior petrosal sinus sampling 

References

  1. 1.
    Magiakou MA, Chrousos GP. Cushing’s syndrome in children and adolescents: current diagnostic and therapeutic strategies. J Endocrinol Investig. 2002;25(2):181–94.CrossRefGoogle Scholar
  2. 2.
    Yordanova G, Martin L, Afshar F, Sabin I, Alusi G, Plowman NP, Riddoch F, Evanson J, Matson M, Grossman AB, Akker SA, Monson JP, Drake WM, Savage MO, Storr HL. Long-term outcomes of children treated for Cushing’s disease: a single center experience. Pituitary. 2016;19(6):612–24.Google Scholar
  3. 3.
    Libuit LG, Karageorgiadis A, Sinaii N, Nguyen May NM, Keil MF, Lodish MB, Stratakis CA. A gender-dependent analysis of Cushing’s disease in childhood: pre- and postoperative follow-up. Clin Endocrinol. 2015;83(1):72–7.  https://doi.org/10.1111/cen.12644; Epub 2015 Feb 2.CrossRefGoogle Scholar
  4. 4.
    Storr HL, Savage MO. Management of endocrine disease: paediatric cushing’s disease. Eur J Endocrinol. 2015;173(1):R35–45.  https://doi.org/10.1530/EJE-15-0013.CrossRefPubMedGoogle Scholar
  5. 5.
    Storr HL, Alexandraki KI, Martin L, Isidori AM, Kaltsas GA, Monson JP, Besser GM, Matson M, Evanson J, Afshar F, Sabin I, Savage MO, Grossman AB. Comparisons in the epidemiology, diagnostic features and cure rate by transsphenoidal surgery between paediatric and adult-onset Cushing’s disease. Eur J Endocrinol. 2011;164(5):667–74.  https://doi.org/10.1530/EJE-10-1120; Epub 2011 Feb 10.CrossRefPubMedGoogle Scholar
  6. 6.
    Lonser RR, Wind JJ, Nieman LK, Weil RJ, DeVroom HL, Oldfield EH. Outcome of surgical treatment of 200 children with Cushing’s disease. J Clin Endocrinol Metab. 2013;98(3):892–901.  https://doi.org/10.1210/jc.2012-3604; Epub 2013 Jan 31.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Chandler WF, Barkan AL, Hollon T, Sakharova A, Sack J, Brahma B, Schteingart DE. Outcome of transsphenoidal surgery for cushing disease: a single-center experience over 32 years. Neurosurgery. 2016;78(2):216–23.  https://doi.org/10.1227/NEU.0000000000001011.CrossRefPubMedGoogle Scholar
  8. 8.
    Batista D, Gennari M, Riar J, Chang R, Keil MF, Oldfield EH, Stratakis CA. An assessment of petrosal sinus sampling for localization of pituitary microadenomas in children with Cushing disease. J Clin Endocrinol Metab. 2006;91(1):221–4; Epub 2005 Oct 11.CrossRefGoogle Scholar
  9. 9.
    Lienhardt A, Grossman AB, Dacie JE, Evanson J, Huebner A, Afshar F, Plowman PN, Besser GM, Savage MO. Relative contributions of inferior petrosal sinus sampling and pituitary imaging in the investigation of children and adolescents with ACTH-dependent Cushing’s syndrome. J Clin Endocrinol Metab. 2001;86(12):5711–4.PubMedGoogle Scholar
  10. 10.
    Lodish M, Dunn SV, Sinaii N, Keil MF, Stratakis CA. Recovery of the hypothalamic-pituitary-adrenal axis in children and adolescents after surgical cure of Cushing’s disease. J Clin Endocrinol Metab. 2012;97(5):1483–91.  https://doi.org/10.1210/jc.2011-2325; Epub 2012 Mar 7.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Valassi E, Biller BM, Swearingen B, Pecori Giraldi F, Losa M, Mortini P, Hayden D, Cavagnini F, Klibanski A. Delayed remission after transsphenoidal surgery in patients with Cushing’s disease. J Clin Endocrinol Metab. 2010;95(2):601–10.  https://doi.org/10.1210/jc.2009-1672; Epub 2010 Jan 15.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Ram Z, Nieman LK, Cutler GB Jr, Chrousos GP, Doppman JL, Oldfield EH. Early repeat surgery for persistent Cushing’s disease. J Neurosurg. 1994;80(1):37–45.CrossRefGoogle Scholar
  13. 13.
    Sughrue ME, Shah JK, Devin JK, Kunwar S, Blevins LS Jr. Utility of the immediate postoperative cortisol concentrations in patients with Cushing’s disease. Neurosurgery. 2010;67(3):688–95.CrossRefGoogle Scholar
  14. 14.
    Leinung MC, Kane LA, Scheithauer BW, Carpenter PC, Laws ERJ, Zimmerman D. Long term follow-up of transsphenoidal surgery for the treatment of Cushing’s disease in childhood. J Clin Endocrinol Metab. 1995;80(8):2475–9.PubMedGoogle Scholar
  15. 15.
    Katavetin P, Cheunsuchon P, Swearingen B, Hedley-Whyte ET, Misra M, Levitsky LL. Review: pituitary adenomas in children and adolescents. J Pediatr Endocrinol Metab. 2010;23(5):427–31.CrossRefGoogle Scholar
  16. 16.
    Kanter AS, Diallo AO, Jane JA Jr, Sheehan JP, Asthagiri AR, Oskouian RJ, Okonkwo DO, Sansur CA, Vance ML, Rogol AD, Laws ER Jr. Single-center experience with pediatric Cushing’s disease. J Neurosurg. 2005;103(5 Suppl):413–20.PubMedGoogle Scholar
  17. 17.
    Knappe UJ, Ludecke DK. Transnasal microsurgery in children and adolescents with Cushing’s disease. Neurosurgery. 39:484–92; discussion 492-483, 1996.Google Scholar
  18. 18.
    Tarapore PE, Sughrue ME, Blevins L, Auguste KI, Gupta N, Kunwar S. Microscopic endonasal transsphenoidal pituitary adenomectomy in the pediatric population. J Neurosurg Pediatr. 2011;7(5):501–9.  https://doi.org/10.3171/2011.2.PEDS10278.CrossRefPubMedGoogle Scholar
  19. 19.
    Storr HL, Drake WM, Evanson J, Matson M, Berney DM, Grossman AB, Akker SA, Monson JP, Alusi G, Savage MO, Sabin I. Endonasal endoscopic transsphenoidal pituitary surgery: early experience and outcome in paediatric Cushing’s disease. Clin Endocrinol. 2014;80(2):270–6.  https://doi.org/10.1111/cen.12275; Epub 2013 Jul 19.CrossRefGoogle Scholar
  20. 20.
    Kiehna EN, Keil M, Lodish M, Stratakis C, Oldfield EH. Pseudotumor cerebri after surgical remission of Cushing’s disease. J Clin Endocrinol Metab. 2010;95(4):1528–32.  https://doi.org/10.1210/jc.2009-2449; Epub 2010 Feb 17.CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Amlashi FG, Swearingen B, Faje AT, Nachtigall LB, Miller KK, Klibanski A, Biller BM, Tritos NA. Accuracy of late-night salivary cortisol in evaluating postoperative remission and recurrence in cushing’s disease. J Clin Endocrinol Metab. 2015;100(10):3770–7.CrossRefGoogle Scholar
  22. 22.
    Patil CG, Veeravagu A, Prevedello DM, Katznelson L, Vance ML, Laws ER Jr. Outcomes after repeat transsphenoidal surgery for recurrent Cushing’s disease. Neurosurgery. 2008;63(2):266–70; discussion 270-1.CrossRefGoogle Scholar
  23. 23.
    Assié G, Bahurel H, Coste J, Silvera S, Kujas M, Dugué MA, Karray F, Dousset B, Bertherat J, Legmann P, Bertagna X. Corticotroph tumor progression after adrenalectomy in Cushing’s Disease: a reappraisal of Nelson’s Syndrome. J Clin Endocrinol Metab. 2007;92(1):172–9; Epub 2006 Oct 24.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of NeurosurgeryMassachusetts General HospitalBostonUSA

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