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Atlas of Sellar and Parasellar Lesions pp 143–153Cite as

Corticotroph (ACTH) Adenomas

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Abstract

Cushing’s disease (CD) caused by an ACTH-secreting pituitary adenoma accounts for 80 % of newly diagnosed cases of Cushing’s syndrome.

Functioning ACTH-staining adenomas comprise approximately 14 % of all surgically resected pituitary adenomas.

The silent ACTH adenomas discussed above are nonfunctioning tumors with positive ACTH immunoreactivity and no clinical evidence of Cushing’s disease or Nelson’s syndrome.

Patients with ACTH hypersecretion may present with Cushing’s syndrome or Nelson’s syndrome.

Cushing’s disease is more common in women than in men (8:1); it typically develops in the third and fourth decades of life.

The disease may also manifest in children and adolescents and comprises a larger proportion of all pituitary adenoma subtypes in pediatric patients than in adults.

Typical symptoms and physical findings in CD include acne, hirsutism or hair loss, weight gain, lipodystrophy, moon facies, skin bruising, abdominal striae, insomnia, amenorrhea, and short-term memory and cognitive deficits.

Among the associated medical conditions are diabetes mellitus, hypertension, osteoporosis, and arthralgia.

Also commonly occurring are psychological disturbances such as anxiety, depression, insomnia, psychosis, or euphoria.

Nelson’s syndrome occurs in patients with ACTH-secreting adenomas who have undergone bilateral adrenalectomy.

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References

  1. Saeger W, Ludecke DK, Buchfelder M, Fahlbusch R, Quabbe HJ, Petersenn S. Pathohistological classification of pituitary tumors: 10 years of experience with the German Pituitary Tumor Registry. Eur J Endocrinol. 2007;156:203–16.

    Article  CAS  PubMed  Google Scholar 

  2. Zada G, Kelly DF, Cohan P, Wang C, Swerdloff R. Endonasal transsphenoidal approach for pituitary adenomas and other sellar lesions: an assessment of efficacy, safety, and patient impressions. J Neurosurg. 2003;98:350–8.

    Article  PubMed  Google Scholar 

  3. Patil CG, Prevedello DM, Lad SP, Vance ML, Thorner MO, Katznelson L, Laws Jr ER. Late recurrences of Cushing’s disease after initial successful transsphenoidal surgery. J Clin Endocrinol Metab. 2008;93:358–62.

    Article  CAS  PubMed  Google Scholar 

  4. Mindermann T, Wilson CB. Age-related and gender-related occurrence of pituitary adenomas. Clin Endocrinol (Oxf). 1994;41:359–64.

    Article  CAS  Google Scholar 

  5. Savage MO, Storr HL, Chan LF, Grossman AB. Diagnosis and treatment of pediatric Cushing’s disease. Pituitary. 2007;10:365–71.

    Article  PubMed  Google Scholar 

  6. Kanter AS, Diallo AO, Jane Jr JA, Sheehan JP, Asthagiri AR, Oskouian RJ, et al. Single-center experience with pediatric Cushing’s disease. J Neurosurg. 2005;103:413–20.

    PubMed  Google Scholar 

  7. Gil-Cardenas A, Herrera MF, Diaz-Polanco A, Rios JM, Pantoja JP. Nelson’s syndrome after bilateral adrenalectomy for Cushing’s disease. Surgery. 2007;141:147–51; discussion 151–2.

    Article  PubMed  Google Scholar 

  8. Banasiak MJ, Malek AR. Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management. Neurosurg Focus. 2007;23:E13.

    Article  PubMed  Google Scholar 

  9. Kelly PA, Samandouras G, Grossman AB, Afshar F, Besser GM, Jenkins PJ. Neurosurgical treatment of Nelson’s syndrome. J Clin Endocrinol Metab. 2002;87:5465–9.

    Article  CAS  PubMed  Google Scholar 

  10. Nieman LK, Biller BM, Findling JW, Newell-Price J, Savage MO, Stewart PM, Montori VM. The diagnosis of Cushing’s syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2008;93:1526–40.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  11. Scheithauer BW, Kovacs KT, Laws Jr ER, Randall RV. Pathology of invasive pituitary tumors with special reference to functional classification. J Neurosurg. 1986;65:733–44.

    Article  CAS  PubMed  Google Scholar 

  12. Jagannathan J, Sheehan JP, Jane Jr JA. Evaluation and management of Cushing syndrome in cases of negative sellar magnetic resonance imaging. Neurosurg Focus. 2007;23:E3.

    Article  PubMed  Google Scholar 

  13. Kucharczyk W, Bishop JE, Plewes DB, Keller MA, George S. Detection of pituitary microadenomas: comparison of dynamic keyhole fast spin-echo, unenhanced, and conventional contrast-enhanced MR imaging. AJR Am J Roentgenol. 1994;163:671–9.

    Article  CAS  PubMed  Google Scholar 

  14. Batista D, Courkoutsakis NA, Oldfield EH, Griffin KJ, Keil M, Patronas NJ, Stratakis CA. Detection of adrenocorticotropin-secreting pituitary adenomas by magnetic resonance imaging in children and adolescents with Cushing disease. J Clin Endocrinol Metab. 2005;90:5134–40.

    Article  CAS  PubMed  Google Scholar 

  15. Lad SP, Patil CG, Laws Jr ER, Katznelson L. The role of inferior petrosal sinus sampling in the diagnostic localization of Cushing’s disease. Neurosurg Focus. 2007;23:E2.

    Google Scholar 

  16. Oldfield EH, Doppman JL, Nieman LK, Chrousos GP, Miller DL, Katz DA, et al. Petrosal sinus sampling with and without corticotropin-releasing hormone for the differential diagnosis of Cushing’s syndrome. N Engl J Med. 1991;325:897–905.

    Article  CAS  PubMed  Google Scholar 

  17. Kaltsas GA, Giannulis MG, Newell-Price JD, Dacie JE, Thakkar C, Afshar F, et al. A critical analysis of the value of simultaneous inferior petrosal sinus sampling in Cushing’s disease and the occult ectopic adrenocorticotropin syndrome. J Clin Endocrinol Metab. 1999;84:487–92.

    CAS  PubMed  Google Scholar 

  18. Bonelli FS, Huston 3rd J, Carpenter PC, Erickson D, Young Jr WF, Meyer FB. Adrenocorticotropic hormone-dependent Cushing’s syndrome: sensitivity and specificity of inferior petrosal sinus sampling. AJNR Am J Neuroradiol. 2000;21:690–6.

    CAS  PubMed  Google Scholar 

  19. Al-Brahim NY, Asa SL. My approach to pathology of the pituitary gland. J Clin Pathol. 2006;59:1245–53.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  20. Meij BP, Lopes MB, Vance ML, Thorner MO, Laws Jr ER. Double pituitary lesions in three patients with Cushing’s disease. Pituitary. 2000;3:159–68.

    Article  CAS  PubMed  Google Scholar 

  21. Jagannathan J, Smith R, DeVroom HL, Vortmeyer AO, Stratakis CA, Nieman LK, Oldfield EH. Outcome of using the histological pseudocapsule as a surgical capsule in Cushing disease. J Neurosurg. 2009;111:531–9.

    Article  PubMed Central  PubMed  Google Scholar 

  22. Esposito F, Dusick JR, Cohan P, Moftakhar P, McArthur D, Wang C, et al. Clinical review: early morning cortisol levels as a predictor of remission after transsphenoidal surgery for Cushing’s disease. J Clin Endocrinol Metab. 2006;91:7–13.

    Article  CAS  PubMed  Google Scholar 

  23. Biller BM, Grossman AB, Stewart PM, Melmed S, Bertagna X, Bertherat J, et al. Treatment of adrenocorticotropin-dependent Cushing’s syndrome: a consensus statement. J Clin Endocrinol Metab. 2008;93:2454–62.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  24. Chen JC, Amar AP, Choi S, Singer P, Couldwell WT, Weiss MH. Transsphenoidal microsurgical treatment of Cushing disease: postoperative assessment of surgical efficacy by application of an overnight low-dose dexamethasone suppression test. J Neurosurg. 2003;98:967–73.

    Article  PubMed  Google Scholar 

  25. Valassi E, Biller BM, Swearingen B, Pecori Giraldi F, Losa M, Mortini P, et al. Delayed remission after transsphenoidal surgery in patients with Cushing’s disease. J Clin Endocrinol Metab. 2010;95:601–10.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  26. Kelly DF. Transsphenoidal surgery for Cushing’s disease: a review of success rates, remission predictors, management of failed surgery, and Nelson’s Syndrome. Neurosurg Focus. 2007;23:E5.

    Article  PubMed  Google Scholar 

  27. Locatelli M, Vance ML, Laws ER. Clinical review: the strategy of immediate reoperation for transsphenoidal surgery for Cushing’s disease. J Clin Endocrinol Metab. 2005;90:5478–82.

    Article  CAS  PubMed  Google Scholar 

  28. Laws ER, Reitmeyer M, Thapar K, Vance ML. Cushing’s disease resulting from pituitary corticotrophic microadenoma. Treatment results from transsphenoidal microsurgery and gamma knife radiosurgery. Neurochirurgie. 2002;48:294–9.

    CAS  PubMed  Google Scholar 

  29. Nakane T, Kuwayama A, Watanabe M, Takahashi T, Kato T, Ichihara K, Kageyama N. Long term results of transsphenoidal adenomectomy in patients with Cushing’s disease. Neurosurgery. 1987;21:218–22.

    Article  CAS  PubMed  Google Scholar 

  30. Engelhardt D, Weber MM. Therapy of Cushing’s syndrome with steroid biosynthesis inhibitors. J Steroid Biochem Mol Biol. 1994;49:261–7.

    Article  CAS  PubMed  Google Scholar 

  31. Basina M, Liu H, Hoffman AR, Feldman D. Successful long-term treatment of Cushing disease with mifepristone (RU486). Endocr Pract. 2012;18:e114–20.

    Article  PubMed  Google Scholar 

  32. Carmichael JD, Fleseriu M. Mifepristone: is there a place in the treatment of Cushing’s disease? Endocrine. 2013;44:20–32.

    Article  CAS  PubMed  Google Scholar 

  33. Fleseriu M, Biller BM, Findling JW, Molitch ME, Schteingart DE, Gross C. Mifepristone, a glucocorticoid receptor antagonist, produces clinical and metabolic benefits in patients with Cushing’s syndrome. J Clin Endocrinol Metab. 2012;97:2039–49.

    Article  CAS  PubMed  Google Scholar 

  34. Boscaro M, Ludlam WH, Atkinson B, Glusman JE, Petersenn S, Reincke M, et al. Treatment of pituitary-dependent Cushing’s disease with the multireceptor ligand somatostatin analog pasireotide (SOM230): a multicenter, phase II trial. J Clin Endocrinol Metab. 2009;94:115–22.

    Article  CAS  PubMed  Google Scholar 

  35. Colao A, De Block C, Gaztambide MS, Kumar S, Seufert J, Casanueva FF. Managing hyperglycemia in patients with Cushing’s disease treated with pasireotide: medical expert recommendations. Pituitary. 2014;17:180–6.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  36. Sheehan JP, Xu Z, Salvetti DJ, Schmitt PJ, Vance ML. Results of gamma knife surgery for Cushing’s disease. J Neurosurg. 2013;119:1486–92.

    Article  PubMed  Google Scholar 

  37. Thompson SK, Hayman AV, Ludlam WH, Deveney CW, Loriaux DL, Sheppard BC. Improved quality of life after bilateral laparoscopic adrenalectomy for Cushing’s disease: a 10-year experience. Ann Surg. 2007;245:790–4.

    Article  PubMed Central  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Neurological Surgery, Keck School of Medicine, University of Southern California, 1200 N State Street, #3300, Los Angeles, CA, 90033, USA

    Gabriel Zada MD, MS

  2. Department of Pathology (Neuropathology), University of Virginia School of Medicine, 1215 Lee Street, Room 3060–HEP, Charlottesville, VA, 22908, USA

    M. Beatriz S. Lopes MD, PhD

  3. Department of Radiology, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA, 02115, USA

    Srinivasan Mukundan Jr PhD, MD

  4. Department of Neurosurgery, Brigham and Women’s Hospital, 15 Francis Street, Boston, MA, 02115, USA

    Edward Laws Jr. MD

Authors
  1. Gabriel Zada MD, MS
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  2. M. Beatriz S. Lopes MD, PhD
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  3. Srinivasan Mukundan Jr PhD, MD
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  4. Edward Laws Jr. MD
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Corresponding author

Correspondence to Gabriel Zada MD, MS .

Editor information

Editors and Affiliations

  1. Department of Neurosurgery, University of Southern California, LOS ANGELES, California, USA

    Gabriel Zada

  2. Department of Pathology, University of Virginia, CHARLOTTESVILLE, Virginia, USA

    M. Beatriz S. Lopes

  3. Department of Radiology, Brigham and Women's Hospital, BOSTON, Massachusetts, USA

    Srinivasan Mukundan Jr.

  4. Department of Neurosurgery, Brigham and Women’s Hospital, BOSTON, Massachusetts, USA

    Edward R. Laws Jr.

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Zada, G., Lopes, M.B.S., Mukundan, S., Laws, E. (2016). Corticotroph (ACTH) Adenomas. In: Zada, G., Lopes, M., Mukundan Jr., S., Laws Jr., E. (eds) Atlas of Sellar and Parasellar Lesions. Springer, Cham. https://doi.org/10.1007/978-3-319-22855-6_13

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  • DOI: https://doi.org/10.1007/978-3-319-22855-6_13

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-22854-9

  • Online ISBN: 978-3-319-22855-6

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