Cushing syndrome

References

Key articles

Sharma ST, Nieman LK, Feelders RA. Cushing's syndrome: epidemiology and developments in disease management. Clin Epidemiol. 2015 Apr 17;7:281-93.Full text  Abstract

Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75.Full text  Abstract

Nieman LK, Biller BM, Findling JW, et al. Treatment of Cushing’s syndrome: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2015 Aug;100(8):2807-31.Full text  Abstract

Feelders RA, Newell-Price J, Pivonello R, et al. Advances in the medical treatment of Cushing's syndrome. Lancet Diabetes Endocrinol. 2019 Apr;7(4):300-12. Abstract

Ritzel K, Beuschlein F, Mickisch A, et al. Clinical review: outcome of bilateral adrenalectomy in Cushing's syndrome: a systematic review. J Clin Endocrinol Metab. 2013 Oct;98(10):3939-48.Full text  Abstract

Reference articles

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20. Hopkins RL, Leinung MC. Exogenous Cushing's syndrome and glucocorticoid withdrawal. Endocrinol Metab Clin North Am. 2005 Jun;34(2):371-84, ix. Abstract

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23. Nieman LK, Biller BM, Findling JW, et al. The diagnosis of Cushing's syndrome: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2008 May;93(5):1526-40.Full text  Abstract

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26. Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75.Full text  Abstract

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28. Galm BP, Qiao N, Klibanski A, et al. Accuracy of laboratory tests for the diagnosis of Cushing syndrome. J Clin Endocrinol Metab. 2020 Jun 1;105(6):2081-94.Full text  Abstract

29. Carroll TB, Raff H, Findling JW. Late-night salivary cortisol for the diagnosis of Cushing syndrome: a meta-analysis. Endocr Pract. 2009 May-Jun;15(4):335-42. Abstract

30. Raff H. Utility of salivary cortisol measurements in Cushing's syndrome and adrenal insufficiency. J Clin Endocrinol Metab. 2009 Oct;94(10):3647-55.Full text  Abstract

31. Zhang Q, Dou J, Gu W, et al. Reassessing the reliability of the salivary cortisol assay for the diagnosis of Cushing syndrome. J Int Med Res. 2013 Oct;41(5):1387-94.Full text  Abstract

32. Petersenn S, Newell-Price J, Findling JW, et al. High variability in baseline urinary free cortisol values in patients with Cushing's disease. Clin Endocrinol (Oxf). 2014 Feb;80(2):261-9.Full text  Abstract

33. Elias PC, Martinez EZ, Barone BF, et al. Late-night salivary cortisol has a better performance than urinary free cortisol in the diagnosis of Cushing's syndrome. J Clin Endocrinol Metab. 2014 Jun;99(6):2045-51.Full text  Abstract

34. Sandouk Z, Johnston P, Bunch D, et al. Variability of late-night salivary cortisol in Cushing disease: a prospective study. J Clin Endocrinol Metab. 2018 Mar 1;103(3):983-90.Full text  Abstract

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36. Carroll BJ, Cassidy F, Naftolowitz D, et al. Pathophysiology of hypercortisolism in depression. Acta Psychiatr Scand Suppl. 2007;(433):90-103. Abstract

37. Raff H, Raff JL, Findling JW. Late-night salivary cortisol as a screening test for Cushing's syndrome. J Clin Endocrinol Metab. 1998 Aug;83(8):2681-6.Full text  Abstract

38. Grober Y, Grober H, Wintermark M, et al. Comparison of MRI techniques for detecting microadenomas in Cushing's disease. J Neurosurg. 2017 Apr 28:1-7. Abstract

39. Newell-Price J, Trainer P, Besser M, et al. The diagnosis and differential diagnosis of Cushing's syndrome and pseudo-Cushing's states. Endocr Rev. 1998 Oct;19(5):647-72.Full text  Abstract

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41. Raff H, Findling JW. A physiologic approach to diagnosis of the Cushing syndrome. Ann Intern Med. 2003 Jun 17;138(12):980-91. Abstract

42. Oldfield EH, Doppman JL, Nieman LK, et al. Petrosal sinus sampling with and without corticotropin-releasing hormone for the differential diagnosis of Cushing's syndrome. N Engl J Med. 1991 Sep 26;325(13):897-905.Full text  Abstract

43. Findling JW, Kehoe ME, Shaker JL, et al. Routine inferior petrosal sinus sampling in the differential diagnosis of adrenocorticotropin (ACTH)-dependent Cushing's syndrome: early recognition of the occult ectopic ACTH syndrome. J Clin Endocrinol Metab. 1991 Aug;73(2):408-13. Abstract

44. Doppman JL, Pass HI, Nieman LK, et al. Detection of ACTH-producing bronchial carcinoid tumors: MR imaging vs CT. AJR Am J Roentgenol. 1991 Jan;156(1):39-43.Full text  Abstract

45. Pacak K, Ilias I, Chen CC, et al. The role of (18)F-fluorodeoxyglucose positron emission tomography and (111)In-diethylenetriaminepentaacetate-D-Phe-pentetreotide scintigraphy in the localization of ectopic adrenocorticotropin-secreting tumors causing Cushing's syndrome. J Clin Endocrinol Metab. 2004 May;89(5):2214-21.Full text  Abstract

46. Panagiotidis E, Alshammari A, Michopoulou S, et al. Comparison of the impact of 68Ga-DOTATATE and 18F-FDG PET/CT on clinical management in patients with neuroendocrine tumors. J Nucl Med. 2017 Jan;58(1):91-6.Full text  Abstract

47. Santhanam P, Taieb D, Giovanella L, et al. PET imaging in ectopic Cushing syndrome: a systematic review. Endocrine. 2015 Nov;50(2):297-305. Abstract

48. Barrio M, Czernin J, Fanti S, et al. The impact of somatostatin receptor-directed PET/CT on the management of patients with neuroendocrine tumor: a systematic review and meta-analysis. J Nucl Med. 2017 May;58(5):756-61. Abstract

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50. Masserini B, Morelli V, Bergamaschi S, et al. The limited role of midnight salivary cortisol levels in the diagnosis of subclinical hypercortisolism in patients with adrenal incidentaloma. Eur J Endocrinol. 2009 Jan;160(1):87-92.Full text  Abstract

51. Pivonello R, De Martino MC, Colao A. How should patients with adrenal incidentalomas be followed up? Lancet Diabetes Endocrinol. 2014 May;2(5):352-4. Abstract

52. Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology clinical practice guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016 Aug;175(2):G1-34.Full text  Abstract

53. Paschou SA, Kandaraki E, Dimitropoulou F, et al. Subclinical Cushing's syndrome in patients with bilateral compared to unilateral adrenal incidentalomas: a systematic review and meta-analysis. Endocrine. 2016 Feb;51(2):225-35. Abstract

54. Scaroni C, Zilio M, Foti M, et al. Glucose metabolism abnormalities in Cushing syndrome: from molecular basis to clinical management. Endocr Rev. 2017 Jun 1;38(3):189-219.Full text  Abstract

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58. Bourdeau I, Bard C, Forget H, et al. Cognitive function and cerebral assessment in patients who have Cushing's syndrome. Endocrinol Metab Clin North Am. 2005 Jun;34(2):357-69;ix. Abstract

59. van der Pas R, Leebeek FW, Hofland LJ, et al. Hypercoagulability in Cushing's syndrome: prevalence, pathogenesis and treatment. Clin Endocrinol (Oxf). 2013 Apr;78(4):481-8.Full text  Abstract

60. Stuijver DJ, van Zaane B, Feelders RA, et al. Incidence of venous thromboembolism in patients with Cushing's syndrome: a multicenter cohort study. J Clin Endocrinol Metab. 2011 Nov;96(11):3525-32.Full text  Abstract

61. Stratakis CA. Skin manifestations of Cushing's syndrome. Rev Endocr Metab Disord. 2016 Sep;17(3):283-6.Full text  Abstract

62. Pecori Giraldi F, Moro M, Cavagnini F, et al. Gender-related differences in the presentation and course of Cushing's disease. J Clin Endocrinol Metab. 2003 Apr;88(4):1554-8.Full text  Abstract

63. Carroll T, Raff H, Findling JW. Late-night salivary cortisol measurement in the diagnosis of Cushing's syndrome. Nat Clin Pract Endocrinol Metab. 2008 Jun;4(6):344-50. Abstract

64. Elamin MB, Murad MH, Mullan R, et al. Accuracy of diagnostic tests for Cushing's syndrome: a systematic review and metaanalyses. J Clin Endocrinol Metab. 2008 May;93(5):1553-62.Full text  Abstract

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66. Rowe NE, Kumar RM, Schieda N, et al. Canadian Urological Association guideline: diagnosis, management, and followup of the incidentally discovered adrenal mass. Can Urol Assoc J. 2023 Feb;17(2):12-24.Full text  Abstract

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68. Nieman LK, Biller BM, Findling JW, et al. Treatment of Cushing’s syndrome: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2015 Aug;100(8):2807-31.Full text  Abstract

69. Swearingen B, Biller BM, Barker FG 2nd, et al. Long-term mortality after transsphenoidal surgery for Cushing disease. Ann Intern Med. 1999 May 18;130(10):821-4. Abstract

70. Hammer GD, Tyrrell JB, Lamborn KR, et al. Transsphenoidal microsurgery for Cushing's disease: initial outcome and long-term results. J Clin Endocrinol Metab. 2004 Dec;89(12):6348-57.Full text  Abstract

71. Pivonello R, De Leo M, Cozzolino A, et al. The treatment of Cushing's disease. Endocr Rev. 2015 Aug;36(4):385-486.Full text  Abstract

72. Broersen LHA, Biermasz NR, van Furth WR, et al. Endoscopic vs. microscopic transsphenoidal surgery for Cushing's disease: a systematic review and meta-analysis. Pituitary. 2018 Oct;21(5):524-34.Full text  Abstract

73. Abu Dabrh AM, Singh Ospina NM, Al Nofal A, et al. Predictors of biochemical remission and recurrence after surgical and radiation treatments of Cushing disease: a systematic disease: a systematic review and meta-analysis. Endocr Pract. 2016 Apr;22(4):466-75. Abstract

74. Patil CG, Prevedello DM, Lad SP, et al. Late recurrences of Cushing's disease after initial successful transsphenoidal surgery. J Clin Endocrinol Metab. 2008 Feb;93(2):358-62.Full text  Abstract

75. Fleseriu M, Hamrahian AH, Hoffman AR, et al; AACE Neuroendocrine and Pituitary Scientific Committee. American Association of Clinical Endocrinologists and American College of Endocrinology Disease State Clinical Review: diagnosis of recurrence in Cushing disease. Endocr Pract. 2016 Dec;22(12):1436-48. Abstract

76. Sughrue ME, Shah JK, Devin JK, et al. Utility of the immediate postoperative cortisol concentrations in patients with Cushing's disease. Neurosurgery. 2010 Sep;67(3):688-95. Abstract

77. Hameed N, Yedinak CG, Brzana J, et al. Remission rate after transsphenoidal surgery in patients with pathologically confirmed Cushing's disease, the role of cortisol, ACTH assessment and immediate reoperation: a large single center experience. Pituitary. 2013 Dec;16(4):452-8. Abstract

78. Danet-Lamasou M, Asselineau J, Perez P, et al. Accuracy of repeated measurements of late-night salivary cortisol to screen for early-stage recurrence of Cushing's disease following pituitary surgery. Clin Endocrinol (Oxf). 2015 Feb;82(2):260-6. Abstract

79. Braun LT, Rubinstein G, Zopp S, et al. Recurrence after pituitary surgery in adult Cushing's disease: a systematic review on diagnosis and treatment. Endocrine. 2020 Nov;70(2):218-31.Full text  Abstract

80. Reincke M, Ritzel K, Osswald A, et al. A critical re-appraisal of bilateral adrenalectomy for ACTH-dependent Cushing's syndrome. Eur J Endocrinol. 2015 Oct;173(4):M23-32.Full text  Abstract

81. Cuevas-Ramos D, Fleseriu M. Treatment of Cushing's disease: a mechanistic update. J Endocrinol. 2014 Nov;223(2):R19-39.Full text  Abstract

82. Friedman RB, Oldfield EH, Nieman LK, et al. Repeat transsphenoidal surgery for Cushing's disease. J Neurosurg. 1989 Oct;71(4):520-7. Abstract

83. Benveniste RJ, King WA, Walsh J, et al. Repeated transsphenoidal surgery to treat recurrent or residual pituitary adenoma. J Neurosurg. 2005 Jun;102(6):1004-12. Abstract

84. Hofmann BM, Hlavac M, Kreutzer J, et al. Surgical treatment of recurrent Cushing's disease. Neurosurgery. 2006 Jun;58(6):1108-18. Abstract

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86. Castinetti F, Nagai M, Dufour H, et al. Gamma knife radiosurgery is a successful adjunctive treatment in Cushing's disease. Eur J Endocrinol. 2007 Jan;156(1):91-8.Full text  Abstract

87. Devin JK, Allen GS, Cmelak AJ, et al. The efficacy of linear accelerator radiosurgery in the management of patients with Cushing's disease. Stereotact Funct Neurosurg. 2004;82(5-6):254-62. Abstract

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89. Starke RM, Williams BJ, Vance ML, et al. Radiation therapy and stereotactic radiosurgery for the treatment of Cushing's disease: an evidence-based review. Curr Opin Endocrinol Diabetes Obes. 2010 Aug;17(4):356-64. Abstract

90. Varlamov EV, McCartney S, Fleseriu M. Functioning pituitary adenomas - current treatment options and emerging medical therapies. Eur Endocrinol. 2019 Apr;15(1):30-40.Full text  Abstract

91. Feelders RA, Newell-Price J, Pivonello R, et al. Advances in the medical treatment of Cushing's syndrome. Lancet Diabetes Endocrinol. 2019 Apr;7(4):300-12. Abstract

92. Hinojosa-Amaya JM, Cuevas-Ramos D, Fleseriu M. Medical management of Cushing's syndrome: current and emerging treatments. Drugs. 2019 Jun;79(9):935-56. Abstract

93. Broersen LHA, Jha M, Biermasz NR, et al. Effectiveness of medical treatment for Cushing's syndrome: a systematic review and meta-analysis. Pituitary. 2018 Dec;21(6):631-41.Full text  Abstract

94. Colao A, Petersenn S, Newell-Price J, et al. A 12-month phase 3 study of pasireotide in Cushing's disease. N Engl J Med. 2012 Mar 8;366(10):914-24.Full text  Abstract

95. Lacroix A, Gu F, Gallardo W, et al. Efficacy and safety of once-monthly pasireotide in Cushing's disease: a 12 month clinical trial. Lancet Diabetes Endocrinol. 2018 Jan;6(1):17-26. Abstract

96. Simeoli C, Auriemma RS, Tortora F, et al. The treatment with pasireotide in Cushing's disease: effects of long-term treatment on tumor mass in the experience of a single center. Endocrine. 2015 Dec;50(3):725-40. Abstract

97. Findling JW, Fleseriu M, Newell-Price J, et al. Late-night salivary cortisol may be valuable for assessing treatment response in patients with Cushing's disease: 12-month, phase III pasireotide study. Endocrine. 2016 Nov;54(2):516-23.Full text  Abstract

98. Trementino L, Michetti G, Angeletti A, et al. A single-center 10-year experience with pasireotide in Cushing's disease: patients' characteristics and outcome. Horm Metab Res. 2016 May;48(5):290-8.Full text  Abstract

99. Petersenn S, Fleseriu M. Pituitary-directed medical therapy in Cushing's disease. Pituitary. 2015 Apr;18(2):238-44. Abstract

100. Palui R, Sahoo J, Kamalanathan S, et al. Effect of cabergoline monotherapy in Cushing's disease: an individual participant data meta-analysis. J Endocrinol Invest. 2018 Dec;41(12):1445-55. Abstract

101. Pivonello R, Fleseriu M, Newell-Price J, et al. Efficacy and safety of osilodrostat in patients with Cushing's disease (LINC 3): a multicentre phase III study with a double-blind, randomised withdrawal phase. Lancet Diabetes Endocrinol. 2020 Sep;8(9):748-61. Abstract

102. Castinetti F, Guignat L, Giraud P, et al. Ketoconazole in Cushing's disease: is it worth a try? J Clin Endocrinol Metab. 2014 May;99(5):1623-30.Full text  Abstract

103. US Food and Drug Administration. FDA drug safety communication: FDA warns that prescribing of Nizoral (ketoconazole) oral tablets for unapproved uses including skin and nail infections continues; linked to patient death. May 2016 [internet publication].Full text

104. Fleseriu M, Castinetti F. Updates on the role of adrenal steroidogenesis inhibitors in Cushing's syndrome: a focus on novel therapies. Pituitary. 2016 Dec;19(6):643-53.Full text  Abstract

105. Fleseriu M, Pivonello R, Elenkova A, et al. Efficacy and safety of levoketoconazole in the treatment of endogenous Cushing's syndrome (SONICS): a phase 3, multicentre, open-label, single-arm trial. Lancet Diabetes Endocrinol. 2019 Nov;7(11):855-65. Abstract

106. Zacharieva SZ, Pivonello R, Elenkova A, et al. MON-332 safety and efficacy of levoketoconazole in the treatment of endogenous Cushing’s syndrome (LOGICS): a double-blind, placebo-controlled, withdrawal study. J Endocr Soc. 2020 May 8;4(suppl 1):MON-332.Full text

107. Fleseriu M, Findling JW, Koch CA, et al. Changes in plasma ACTH levels and corticotroph tumor size in patients with Cushing's disease during long-term treatment with the glucocorticoid receptor antagonist mifepristone. J Clin Endocrinol Metab. 2014 Oct;99(10):3718-27.Full text  Abstract

108. Fleseriu M, Biller BM, Findling JW, et al; SEISMIC Study Investigators. Mifepristone, a glucocorticoid receptor antagonist, produces clinical and metabolic benefits in patients with Cushing's syndrome. J Clin Endocrinol Metab. 2012 Jun;97(6):2039-49.Full text  Abstract

109. Neychev V, Steinberg SM, Yang L, et al. Long-term outcome of bilateral laparoscopic adrenalectomy measured by disease-specific questionnaire in a unique group of patients with Cushing's syndrome. Ann Surg Oncol. 2015 Dec;22 Suppl 3:S699-706. Abstract

110. Ritzel K, Beuschlein F, Mickisch A, et al. Clinical review: outcome of bilateral adrenalectomy in Cushing's syndrome: a systematic review. J Clin Endocrinol Metab. 2013 Oct;98(10):3939-48.Full text  Abstract

111. Fleseriu M, Hashim IA, Karavitaki N, et al. Hormonal replacement in hypopituitarism in adults: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2016 Nov;101(11):3888-921.Full text  Abstract

112. Bancos I, Alahdab F, Crowley RK, et al. Therapy of endocrine disease: improvement of cardiovascular risk factors after adrenalectomy in patients with adrenal tumors and subclinical Cushing's syndrome: a systematic review and meta-analysis. Eur J Endocrinol. 2016 Dec;175(6):R283-95.Full text  Abstract

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114. Fleseriu M, Petersenn S. Medical management of Cushing's disease: what is the future? Pituitary. 2012 Sep;15(3):330-41.Full text  Abstract

115. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text

116. Yip L, Duh QY, Wachtel H, et al. American Association of Endocrine Surgeons guidelines for adrenalectomy: executive summary. JAMA Surg. 2022 Oct 1;157(10):870-7. Abstract

117. De Tommasi C, Vance ML, Okonkwo DO, et al. Surgical management of adrenocorticotropic hormone-secreting macroadenomas: outcome and challenges in patients with Cushing's disease or Nelson's syndrome. J Neurosurg. 2005 Nov;103(5):825-30. Abstract

118. Faggiano A, Pivonello R, Spiezia S, et al. Cardiovascular risk factors and common carotid artery caliber and stiffness in patients with Cushing's disease during active disease and 1 year after disease remission. J Clin Endocrinol Metab. 2003 Jun;88(6):2527-33.Full text  Abstract

119. Van der Klaauw AA, Kars M, Biermasz NR, et al. Disease-specific impairments in quality of life during long-term follow-up of patients with different pituitary adenomas. Clin Endocrinol (Oxf). 2008 Nov;69(5):775-84. Abstract

120. Knoble N, Nayroles G, Cheng C, et al. Illustration of patient-reported outcome challenges and solutions in rare diseases: a systematic review in Cushing's syndrome. Orphanet J Rare Dis. 2018 Dec 19;13(1):228.Full text  Abstract

121. Petersenn S, Beckers A, Ferone D, et al. Therapy of endocrine disease: outcomes in patients with Cushing's disease undergoing transsphenoidal surgery: systematic review assessing criteria used to define remission and recurrence. Eur J Endocrinol. 2015 June;172(6):R227-39.Full text  Abstract

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123. Qiao N. Outcome of endoscopic vs microsurgical transsphenoidal resection for Cushing's disease. Endocr Connect. 2018 Jan;7(1):R26-37.Full text  Abstract

124. Dhandapani S, Narayanan R, Jayant SS, et al. Endonasal endoscopic versus microscopic transsphenoidal surgery in pituitary tumors among the young: a comparative study & meta-analysis. Clin Neurol Neurosurg. 2021 Jan;200:106411. Abstract

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