Skip to main content
SpringerLink
Log in

Adrenocorticotrophic hormone-dependent Cushing’s syndrome

  • Chapter
Endocrine Neoplasms

Part of the book series: Cancer Treatment and Research ((CTAR,volume 89))

Abstract

Cushing’s syndrome is the state that results from prolonged exposure of tissues to excess glucocorticoids. At the beginning of the century, Harvey Cushing recognized that Cushing’s syndrome could result from tumors arising from either the anterior pituitary (basophilic adenomata) or the adrenal cortex [1,2]. This observation set the basis for our current classification of Cushing’s syndrome into adrenocorticotrophic hormone (ACTH)-dependent and ACTH-independent forms. The former, in addition to pituitary adenomas, includes nonpituitary tumors that secrete ACTH (ectopic ACTH syndrome) or, rarely, corticotropin-releasing hormone (CRH; ectopic CRH syndrome). In the early 1950s, when glucocorticoids were first employed in the treatment of rheumatoid arthritis, the iatrogenic form of Cushing’s syndrome appeared. As the use of glucocorticoids was extended to many immunological, hematologic, renal, and other diseases, the prevalence of exogenous Cushing’s syndrome far exceeded that of the endogenous forms. More recently, factitious use of glucocorticoids was also reported [3].

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 259.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 329.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 329.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Cushing H. 1912. The Pituitary Body and its Disorders. Philadelphia: JB Lippincott, p 219.

    Google Scholar 

  2. Cushing H. 1932. The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism). Bull John Hopkins Hosp 50:137–195.

    Google Scholar 

  3. Cizza G, Nieman LK, Doppman JL, Passaro FS, Czerwiec FS, Chrousos GP, Cutler GB. 1996. Factitious Cushing syndrome. J Clin Endocrinol Metab 81:3573–3577.

    Article  PubMed  CAS  Google Scholar 

  4. Magiakou MA, Mastorakos G, Oldfield EA, Gomez MT, Doppman JL, Cutler GB Jr, Nieman LK, Chrousos GP. 1994. Cushing syndrome in children and adolescents: Presentation, diagnosis and therapy. N Engl J Med 331:629–636.

    Article  PubMed  CAS  Google Scholar 

  5. Magiakou MA, Mastorakos G, Chrousos GP. 1994. Final stature in patients with endogenous Cushing syndrome. J Clin Endocrinol Metab 79:1082–1085.

    Article  PubMed  CAS  Google Scholar 

  6. Carey RM, Varna SK, Drake CR, Thorner MO, Kovacs K, Rivier J, Vale W. 1984. Ectopic secretion of corticotropin-releasing factor as a cause of Cushing’s syndrome. N Engl J Med 311:13–20.

    Article  PubMed  CAS  Google Scholar 

  7. Auchus RJ, Mastorakos G, Friedman TC, Chrousos GP. 1994. Corticotropin releasing hormone production by a small cell carcinoma in a patient with ACTH-dependent Cushing syndrome. J Endocrinol Invest 17:447–452.

    PubMed  CAS  Google Scholar 

  8. Buckley N, Bates AS, Broome JC, Strange RC, Perrett CW, Burke CW, Clayton RN. 1994. P53 protein accumulates in Cushing’s adenomas and invasive non-functional adenomas. J Clin Endocrinol Metab 79:1518–1521.

    Article  Google Scholar 

  9. Karl M, Von Wichert G, Kempter E, Katz DA, Reincke M, Monig H, Ali IU, Stratakis CA, Oldfield EH, Chrousos GP, Schulte HM. 1996. Nelson’s syndrome associated with a somatic frame shift mutation in the glucocorticoid receptor gene. J Clin Endocrinol Metab 81:124–129.

    Article  PubMed  CAS  Google Scholar 

  10. Tsigos C, Chrousos GP. 1995. Cushing’s syndrome. Curr Opin Endocrinol Diabetes 2:203–213.

    Article  Google Scholar 

  11. Gomez MT, Malozowski S, Winterer J, Chrousos GP. 1991. Urinary free cortisol values in normal children and adolescents. J Pediatr 118:256–258.

    Article  PubMed  CAS  Google Scholar 

  12. Contreras LN, Hanes S, Tyrrell JB. 1986. Urinary free cortisol in the assessment of pituitaryadrenal function. Utility of 24-hour and spot determinations. J Clin Endocrinol Metab 62:965–969.

    Article  PubMed  CAS  Google Scholar 

  13. Pavlatos FC, Smilo RP, Forsham PH. 1965. A rapid screening test for Cushing’s syndrome. JAMA 193:720–723.

    Article  PubMed  CAS  Google Scholar 

  14. Tsigos C, Chrousos GP. 1994. Physiology of the hypothalamic pituitary-adrenal axis in health and dysregulation in psychiatric and autoimmune disorders. Endocrinol Metab Clin 23:451–466.

    CAS  Google Scholar 

  15. Tsigos C, Chrousos GP. 1995. Stress, endocrine manifestations and diseases. In Cooper GL, ed. Handbook of Stress Medicine. Boca Raton, FL: CRC Press, pp 61–65.

    Google Scholar 

  16. Gold PW, Loriaux DL, Roy A, Kling MA, Calabrese JR, Kellner CH, Nieman LK, Post RM, Pickar D, Gallucci W, Avgerinos P, Paul S, Oldfield EH, Cutler GB Jr, Chrousos GP. 1986. Responses to corticotropin-releasing hormone in the hypercortosolism of depression and Cushing’s disease: Pathophysiologic and diagnostic implications. N Engl J Med 314:1329–1335.

    Article  PubMed  CAS  Google Scholar 

  17. Yanovski JA, Cutler GB Jr, Chrousos GP, Nieman LK. 1993. Corticotropin-releasing hormone stimulation following low dose dexamethasone administration. A new test to distinguish Cushing’s syndrome from pseudo-Cushing’s states. JAMA 269:2232–2238.

    Article  PubMed  CAS  Google Scholar 

  18. Orth DN. 1991. Differential diagnosis of Cushing’s syndrome. N Engl J Med 325:957.

    Article  PubMed  CAS  Google Scholar 

  19. White A, Clark AJL. 1993. The cellular and molecular basis of the ectopic ACTH syndrome. Clin Endocrinol 39:131–141.

    Article  CAS  Google Scholar 

  20. Chrousos GP, Schulte HM, Oldfield EH, Gold PW, Cutler GB Jr, Loriaux DL. 1984. The corticotropin-releasing factor stimulation test: An aid in the evaluation of patients with Cushing’s syndrome. N Engl J Med 310:622–627.

    Article  PubMed  CAS  Google Scholar 

  21. Nieman LK, Oldfield EH, Wesley R, Chrousos GP, Loriaux DX, Cutler GB Jr. 1993. A simplified morning ovine corticotropin-releasing hormone stimulation test for the differential diagnosis of ACTH dependent Cushing’s syndrome. J Clin Endocrinol Metab 77:1308–1312.

    Article  PubMed  CAS  Google Scholar 

  22. Liddle GW. 1960. Tests of pituitary-adrenal suppressibility in the diagnosis of Cushing’s syndrome. J Clin Endocrinol Metab 20:1539–1561.

    Article  PubMed  CAS  Google Scholar 

  23. Flack MR, Oldfield EH, Cutler GB Jr, Nieman LK, Loriaux DX. 1991. Urine free cortisol in the high-dose dexamethasone suppression test for the differential diagnosis of the Cushing syndrome. Ann Intern Med 116:211–217.

    Google Scholar 

  24. Tyrell JB, Findling JW, Aron DC, Fitzerald PA, Forsham PH. 1986. An overnight high-dose dexamethasone suppression test for rapid differential diagnosis of Cushing’s syndrome. Ann Intern Med 104:180–186.

    Google Scholar 

  25. Dichek HL, Nieman LK, Oldfield EH, Pass HI, Malley JD, Cutler GB Jr. 1994. A comparison of the standard high dose dexamethasone suppression test and the overnight 8-mg dexamethasone suppression test for the differential diagnosis of adrenocorticotropin-dependent Cushing’s syndrome. J Clin Endocrinol Metab 78:418–422.

    Article  PubMed  CAS  Google Scholar 

  26. Doppman JF, Reinig JW, Dwyer A, Frank JA, Loriaux DL, Keiser H, Norton JA. 1987. Differentiation of adrenal masses by magnetic resonance imaging. Surgery 102:1018–1026.

    PubMed  CAS  Google Scholar 

  27. Rufini V, Salentnich I, Troncone L. 1992. Radiocholesterol scintigraphy in Cushing’s syndrome. Rays 17:40–48.

    PubMed  CAS  Google Scholar 

  28. Doppman JL, Frank JA, Dwyer AJ, Oldfield EH, Miller DL, Nieman LK, Chrousos GP, Cutler GB Jr, Loriaux DL. 1988. Gadolinium-DTPA enhanced imaging of ACTH-secreting microadenomas of the pituitary gland: Correlation of MR appearance with surgical findings. J Comput Assist Tomogr 12:728–735.

    Article  PubMed  CAS  Google Scholar 

  29. Doppman JL, Nieman L, Miller DL, Pass HI, Chung R, Cutler GB Jr, Schaaf M, Chrousos GP, Norton JA, Zirrsman HA, Oldfield EH, Loriaux DL. Ectopic adrenocorticotropic hormone syndrome: Localization studies in 28 patients. Radiology 172:115–124.

    Google Scholar 

  30. Leinung MC, Young WF Jr, Whitaker MD, Scheithauer BW, Tvastek VF, Kvols LK. 1990. Diagnosis of corticotropin-producing bronchial carcinoid tumors causing Cushing’s syndrome. Mayo Clin Proc 65:1314–1321.

    PubMed  CAS  Google Scholar 

  31. Doppman JL, Pass HI, Nieman LK, Findling TW, Dwyer AJ, Fenerstein IM, Ling A, Travis WD, Cutler GB Jr, Chrousos GP, Loriaux DL. 1991. Detection of ACTH-producing bronchial carcinoid tumors: MR imaging vs. CT. Am J Radiol 156:39–43.

    CAS  Google Scholar 

  32. Limper AH, Carpenter PC, Scheithauser B, Staats BA. 1992. The Cushing syndrome induced by bronchial carcinoid tumors. Ann Intern Med 117:209–214.

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  34. Miller DL, Doppman JL, Peterman SB, Nieman LK, Oldfield EH, Chang R. 1992. Neurologic complications of petrosal sinus sampling. Radiology 185:143–147.

    PubMed  CAS  Google Scholar 

  35. Vagnucci AH, Evans E. 1986. Cushing’s disease with intermittent hypercortisolism. Am J Med 80:83–88.

    Article  PubMed  CAS  Google Scholar 

  36. Sakiyama R, Ashcraft MW, Van Herle AJ. 1984. Cyclic Cushing’s syndrome. Am J Med 77:944–946.

    Article  PubMed  CAS  Google Scholar 

  37. Doppman JL, Pass HI, Nieman LK, Miller DL, Chang R, Cutler GB, Chrousos GP, Jaffe GS, Norton JA. 1992. Corticotropin-secreting carcinoid tumors of the thymus: Diagnostic unreliability of thymic venous sampling. Radiology 184:71–74.

    PubMed  CAS  Google Scholar 

  38. Sasaki A, Shinkawa O, Margioris AN, et al. 1987. Immunoreactive corticotropin-releasing hormone in human plasma during pregnancy, labor and delivery. J Clin Endocrinol Metab 64:224–229.

    Article  PubMed  CAS  Google Scholar 

  39. Buescher MA, McClamrock HD, Adashi EY. 1992. Cushing syndrome in pregnancy. Obstet Gynecol 79:130–137.

    PubMed  CAS  Google Scholar 

  40. Mampalam TJ, Tyrrell JB, Wilson CB. 1988. Transphenoidal microsurgery for Cushing’s disease. A report of 216 cases. Ann Intern Med 109:487–493.

    PubMed  CAS  Google Scholar 

  41. Oldfield EH, Chrousos GP, Schulte HM, Loriaux DL, Schaaf M, Doppman J. 1985. Preoperative lateralization of ACTH-secreting pituitary microadenomas by bilateral and simultaneous inferior petrosal venous sinus sampling. N Engl J Med 312:100–103.

    Article  PubMed  CAS  Google Scholar 

  42. Ram Z, Nieman LK, Cutler GB Jr, Chrousos GP, Doppman J, Oldfield EH. 1994. Early repeat surgery for persistent Cushing’s disease. J Neurosurg 80:37–45.

    Article  PubMed  CAS  Google Scholar 

  43. Avgerinos PC, Chrousos GP, Nieman LK, Oldfield EH, Loriaux DL, Cutler GB Jr. 1987. The corticotropin-releasing hormone test in the postoperative evaluation of patients with Cushing’s syndrome. J Clin Endocrinol Metab 65:906–913.

    Article  PubMed  CAS  Google Scholar 

  44. Luton JP, Mahoudeau JA, Bouchard P, Thieblot P, Hautecouverture M, Simon D, Laudat MH, Touitou Y, Bricaire H. 1979. Treatment of Cushing’s disease by o, p’DDD. Survey of 62 cases. N Engl J Med 300:459–464.

    Article  PubMed  CAS  Google Scholar 

  45. Zeiger MA, Fraker DL, Pass HI, Nieman LK, Cutler GB, Chrousos GP, Norton JA. 1993. Effective reversibility of the signs and symptoms of hypercortisolism by bilateral adrenalectomy. Surgery 114:1138–1143.

    PubMed  CAS  Google Scholar 

  46. Nelson DH, Meakin JW, Thorn GW. 1960. ACTH-producing pituitary tumors following adrenalectomy for Cushing’s syndrome. Ann Intern Med 52:560–569.

    PubMed  CAS  Google Scholar 

  47. Davila DG, Dunn WF, Tazelaar HD, Pairolero PC. 1993. Bronchial carcinoid tumors. Mayo Clinic Proc 68:795–803.

    CAS  Google Scholar 

  48. Doppman JL, Nieman LK, Cutler GB Jr, Chrousos GP, Fraker DL, Norton JA, Jensen RT. 1994. Adrenocorticotropin hormone-secreting islet cell tumors: Are they always malignant? Radiology 190:59–64.

    PubMed  CAS  Google Scholar 

  49. Miller JW, Crapo L. 1993. The medical treatment of Cushing’s syndrome. Endocr Rev 14:443–458.

    PubMed  CAS  Google Scholar 

  50. Doherty G, Nieman LK, Cutler GB Jr, Chrousos GP, Norton J. 1990. Time to recovery of the hypothalamic-pituitary-adrenal axis after curative resection of adrenal tumors in patients with Cushing’s syndrome. Surgery 108:1085–1090.

    PubMed  CAS  Google Scholar 

Download references

Authors
  1. Giovanni Cizza
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. George P. Chrousos
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Laboratory of Endocrine Oncology Massachusetts General Hospital, Harvard Medical School, Jackson 1021, Boston, MA, 02114, USA

    Andrew Arnold M.D. (Chief, Associate Professor of Medicine) (Chief, Associate Professor of Medicine)

Rights and permissions

Reprints and permissions

Copyright information

© 1997 Springer Science+Business Media New York

About this chapter

Cite this chapter

Cizza, G., Chrousos, G.P. (1997). Adrenocorticotrophic hormone-dependent Cushing’s syndrome. In: Arnold, A. (eds) Endocrine Neoplasms. Cancer Treatment and Research, vol 89. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-6355-6_2

Download citation

  • DOI: https://doi.org/10.1007/978-1-4615-6355-6_2

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-7919-5

  • Online ISBN: 978-1-4615-6355-6

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation