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Cushing's Disease: A Review

Cushing's Disease: A Review Abstract • Cushing's syndrome continues to tax the most discerning clinician. I review pituitary-dependent adrenal hyperplasia (Cushing's disease), including recent experiences with Cushing's disease at Duke University, Durham, NC, and relate these observations to the current ideas as to pathophysiology, etiology, and management of Cushing's disease. Transsphenoidal microsurgery (TPS) performed by an experienced neurosurgeon offers selective removal of corticotropin (ACTH)-secreting adenoma, immediately cures the hypercortisolism, preserves pituitary function, and is associated with minimal morbidity. Postoperative hypoadrenalism appears to be the best marker of surgical cure. Transsphenoidal surgery has revolutionized our thoughts as to etiology and treatment of Cushing's disease, yet failures with TPS and uncertainty of recurrences leave room for radiotherapy, adrenalectomy, and adjunctive drug therapy in the management of this entity. (Arch Intern Med 1985;145:1106-1111) References 1. Aron DC, Tyrrell JB, Fitzgerald PA, et al: Cushing's syndrome: Problems in diagnosis. Medicine 1981;60:25-35.Crossref 2. Krieger DT: Physiopathology of Cushing's disease. Endocr Rev 1983; 4:22-43.Crossref 3. Cushing H: The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism). Bull Johns Hopkins Hosp 1932;50:137-195. 4. Tyrrell JB, Brooks RM, Fitzgerald PA, et al: Cushing's disease: Selective resection of pituitary microadenomas. N Engl J Med 1978;298: 753-758.Crossref 5. Salassa RM, Laws ER Jr, Carpenter PC, et al: Transsphenoidal removal of pituitary microadenoma in Cushing's disease. Mayo Clin Proc 1978;53:24-28. 6. Bigos ST, Somma M, Rasio E, et al: Cushing's disease: Management by transsphenoidal pituitary microsurgery. J Clin Endocrinol Metabol 1980; 50:348-354.Crossref 7. Gold E: The Cushing's syndromes: Changing views of diagnosis and treatment. Ann Intern Med 1979;90:829-844.Crossref 8. Burch W: A survey of results with transsphenoidal surgery in Cushing's disease. N Engl J Med 1983;308:103-104.Crossref 9. Kuwayama A, Kageyama N, Nakane T, et al: Anterior pituitary function after transsphenoidal selective adenomectomy in patients with Cushing's disease. J Clin Endocrinol Metabol 1981;53:165-173.Crossref 10. Fitzgerald PA, Aron DC, Findling JW, et al: Cushing's disease: Transient secondary adrenal insufficiency after selective removal of pituitary microadenomas: Evidence for a pituitary origin. J Clin Endocrinol Metabol 1982;54:413-422.Crossref 11. Orth DN, DeBold CR, DeCherney GS, et al: Pituitary microadenomas causing Cushing's disease respond to corticotropin-releasing factor. J Clin Endocrinol Metabol 1982;55:1017-1018.Crossref 12. Lamberts SWJ, Birkenhager JC: The mechanism of the suppressive action of bromocriptine on ACTH secretion in patients with Cushing's and Nelson's syndrome. J Clin Endocrinol Metabol 1980;51:307-315.Crossref 13. Lamberts SWJ, Stefanko SZ, de Lange SA, et al: Failure of clinical remission after transsphenoidal removal of a microadenoma in a patient with Cushing's disease: Multiple hyperplastic and adenomatous cell nests in surrounding pituitary tissue. J Clin Endocrinol Metabol 1980;50:793-795.Crossref 14. Lankford HV, Tucker HSG, Blackard WG: A cyproheptadine-reversible defect in ACTH control persisting after removal of the pituitary tumor in Cushing's disease. N Engl J Med 1981;305:1244-1248.Crossref 15. Fehm HL, Steck R, Hohnoser J, et al: Influence of neuroactive drugs on corticosteroid feedback regulation of ACTH secretion in man. Horm Metab Res 1983;15:29-32.Crossref 16. Suda T, Tozawa F, Mouri T, et al: Effects of cyproheptadine, reserpine, and synthetic corticotropin-releasing factor on pituitary glands from patients with Cushing's disease. J Clin Endocrinol Metabol 1983;56:1094-1099.Crossref 17. McNicol AM, Thomson H, Stewart CJR: The corticotropic cells of the canine pituitary gland in pituitary-dependent hyperadrenocorticism. J Endocrinol 1983;96:303-309.Crossref 18. Moore JN, Steiss J, Nicholson WE, et al: A pituitary adrenocorticotropin-dependent Cushing's syndrome in the horse. Endocrinology 1979;104:576-582.Crossref 19. Burch WM: Diagnosis and treatment of Cushing's disease at Duke University (1977-1982). NC Med J 1983;44:293-296. 20. Boggan JE, Tyrrell JB, Wilson CB: Transsphenoidal microsurgical management of Cushing's disease. J Neurosurg 1983;59:195-200.Crossref 21. Hardy J: Cushing's disease: 50 years later. Can J Neurol Sci 1982;9: 375-380. 22. Orth DN: Cushing's syndrome , in Krieger DT, Bardin CW (eds): Current Therapy in Endocrinology 1983-1984 . Burlington, Ontario, B. C. Decker, 1983, pp 119-127. 23. Pont A, Gutierrez-Hartman A: Cushing's disease: Recurrence after a surgically induced remission. Arch Intern Med 1979;139:938-940.Crossref 24. Ludecke D, Kautzky R, Saeger W, et al: Selective removal of hypersecreting pituitary adenomas. Acta Neurochir 1976;35:27-42.Crossref 25. Strott CA, Nugent CA, Tyler FH: Cushing's syndrome caused by bronchial adenomas. Am J Med 1968;44:97-103.Crossref 26. Aron DC, Findling JW, Fitzgerald PA, et al: Cushing's syndrome: Problems in management. Endocr Rev 1982;3:229-244.Crossref 27. Doppman JL, Oldfield E, Krudy AG, et al: Petrosal sinus sampling for Cushing syndrome: Anatomical and technical considerations. Radiology 1984;150:99-103.Crossref 28. Prinz RA, Brooks MH, Lawrence AM, et al: The continued importance of adrenalectomy in the treatment of Cushing's disease. Arch Surg 1979;114:481-484.Crossref 29. Delaney JP, Solomkim JS, Jacabson ME, et al: Surgical management of Cushing's syndrome. Surgery 1978;84:465-470. 30. Nelson DH, Meakin JW, Thorn GW: ACTH-producing pituitary tumors following adrenalectomy for Cushing's syndrome. Ann Intern Med 1960;52:560-569.Crossref 31. Moore TJ, Dluhy RG, Williams GH, et al: Nelson's syndrome: Frequency, prognosis, and effect of prior irradiation. Ann Intern Med 1976;85: 731-734.Crossref 32. Jennings AS, Liddle GW, Orth DN: Results of treating childhood Cushing's disease with pituitary irradiation. N Engl J Med 1977;297:957-962.Crossref 33. Orth DN, Liddle GW: Results of treatment in 108 patients with Cushing's syndrome. N Engl J Med 1971;285:243-247.Crossref 34. Barnett AH, Livesey JH, Friday K, et al: Comparison of preoperative and postoperative ACTH concentrations after bilateral adrenalectomy in Cushing's disease. Clin Endocrinol 1983;18:301-305.Crossref 35. Styne DM, Grumbach MM, Kaplan SL, et al: Treatment of Cushing's disease in childhood and adolescence by transsphenoidal microadenomectomy. N Engl J Med 1984;310:889-893.Crossref 36. McArthur RG, Hayles AB, Salassa RM: Childhood Cushing disease: Results of bilateral adrenalectomy. J Pediatr 1979;95:214-219.Crossref 37. Krieger DT, Amorosa L, Linick F: Cyproheptadine-induced remission of Cushing's disease. N Engl J Med 1975;293:893-896.Crossref 38. Krieger DT: Cyproheptadine: Drug therapy for Cushing's disease , in Muller EE (ed): Neuroactive Drugs in Endocrinology . Amsterdam, Elsevier-North Holland, 1980, pp 361-370. 39. Aronin N, Krieger DT: Sustained remission of Nelson's syndrome after stopping cyproheptadine treatment. N Engl J Med 1980;302:453-455.Crossref 40. Hsu T-H, Gann DS, Tasn K-W, et al: Cyproheptadine in the control of Cushing's disease. Johns Hopkins Med J 1981;149:77-83. 41. Lamberts SWJ, de Lange SA, Stefanko SZ: Adrenocorticotropinsecreting pituitary adenomas originate from the anterior or the intermediate lobe in Cushing's disease: Differences in the regulation of hormone secretion. J Clin Endocrinol Metabol 1982;54:286-291.Crossref 42. Miura K, Aida M, Mihara A, et al: Treatment of Cushing's disease with reserpine and pituitary irradiation. J Clin Endocrinol Metabol 1975;41:511-526.Crossref 43. Dornhorst A, Jenkins JS, Lamberts SWJ, et al; The evaluation of sodium valproate in the treatment of Nelson's syndrome. J Clin Endocrinol Metabol 1983;56:985-991.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Cushing's Disease: A Review

Archives of Internal Medicine , Volume 145 (6) – Jun 1, 1985

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Publisher
American Medical Association
Copyright
Copyright © 1985 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1985.00360060174027
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Abstract

Abstract • Cushing's syndrome continues to tax the most discerning clinician. I review pituitary-dependent adrenal hyperplasia (Cushing's disease), including recent experiences with Cushing's disease at Duke University, Durham, NC, and relate these observations to the current ideas as to pathophysiology, etiology, and management of Cushing's disease. Transsphenoidal microsurgery (TPS) performed by an experienced neurosurgeon offers selective removal of corticotropin (ACTH)-secreting adenoma, immediately cures the hypercortisolism, preserves pituitary function, and is associated with minimal morbidity. Postoperative hypoadrenalism appears to be the best marker of surgical cure. Transsphenoidal surgery has revolutionized our thoughts as to etiology and treatment of Cushing's disease, yet failures with TPS and uncertainty of recurrences leave room for radiotherapy, adrenalectomy, and adjunctive drug therapy in the management of this entity. (Arch Intern Med 1985;145:1106-1111) References 1. Aron DC, Tyrrell JB, Fitzgerald PA, et al: Cushing's syndrome: Problems in diagnosis. Medicine 1981;60:25-35.Crossref 2. Krieger DT: Physiopathology of Cushing's disease. Endocr Rev 1983; 4:22-43.Crossref 3. Cushing H: The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism). Bull Johns Hopkins Hosp 1932;50:137-195. 4. Tyrrell JB, Brooks RM, Fitzgerald PA, et al: Cushing's disease: Selective resection of pituitary microadenomas. N Engl J Med 1978;298: 753-758.Crossref 5. Salassa RM, Laws ER Jr, Carpenter PC, et al: Transsphenoidal removal of pituitary microadenoma in Cushing's disease. Mayo Clin Proc 1978;53:24-28. 6. Bigos ST, Somma M, Rasio E, et al: Cushing's disease: Management by transsphenoidal pituitary microsurgery. J Clin Endocrinol Metabol 1980; 50:348-354.Crossref 7. Gold E: The Cushing's syndromes: Changing views of diagnosis and treatment. Ann Intern Med 1979;90:829-844.Crossref 8. Burch W: A survey of results with transsphenoidal surgery in Cushing's disease. N Engl J Med 1983;308:103-104.Crossref 9. Kuwayama A, Kageyama N, Nakane T, et al: Anterior pituitary function after transsphenoidal selective adenomectomy in patients with Cushing's disease. J Clin Endocrinol Metabol 1981;53:165-173.Crossref 10. Fitzgerald PA, Aron DC, Findling JW, et al: Cushing's disease: Transient secondary adrenal insufficiency after selective removal of pituitary microadenomas: Evidence for a pituitary origin. J Clin Endocrinol Metabol 1982;54:413-422.Crossref 11. Orth DN, DeBold CR, DeCherney GS, et al: Pituitary microadenomas causing Cushing's disease respond to corticotropin-releasing factor. J Clin Endocrinol Metabol 1982;55:1017-1018.Crossref 12. Lamberts SWJ, Birkenhager JC: The mechanism of the suppressive action of bromocriptine on ACTH secretion in patients with Cushing's and Nelson's syndrome. J Clin Endocrinol Metabol 1980;51:307-315.Crossref 13. Lamberts SWJ, Stefanko SZ, de Lange SA, et al: Failure of clinical remission after transsphenoidal removal of a microadenoma in a patient with Cushing's disease: Multiple hyperplastic and adenomatous cell nests in surrounding pituitary tissue. J Clin Endocrinol Metabol 1980;50:793-795.Crossref 14. Lankford HV, Tucker HSG, Blackard WG: A cyproheptadine-reversible defect in ACTH control persisting after removal of the pituitary tumor in Cushing's disease. N Engl J Med 1981;305:1244-1248.Crossref 15. Fehm HL, Steck R, Hohnoser J, et al: Influence of neuroactive drugs on corticosteroid feedback regulation of ACTH secretion in man. Horm Metab Res 1983;15:29-32.Crossref 16. Suda T, Tozawa F, Mouri T, et al: Effects of cyproheptadine, reserpine, and synthetic corticotropin-releasing factor on pituitary glands from patients with Cushing's disease. J Clin Endocrinol Metabol 1983;56:1094-1099.Crossref 17. McNicol AM, Thomson H, Stewart CJR: The corticotropic cells of the canine pituitary gland in pituitary-dependent hyperadrenocorticism. J Endocrinol 1983;96:303-309.Crossref 18. Moore JN, Steiss J, Nicholson WE, et al: A pituitary adrenocorticotropin-dependent Cushing's syndrome in the horse. Endocrinology 1979;104:576-582.Crossref 19. Burch WM: Diagnosis and treatment of Cushing's disease at Duke University (1977-1982). NC Med J 1983;44:293-296. 20. Boggan JE, Tyrrell JB, Wilson CB: Transsphenoidal microsurgical management of Cushing's disease. J Neurosurg 1983;59:195-200.Crossref 21. Hardy J: Cushing's disease: 50 years later. Can J Neurol Sci 1982;9: 375-380. 22. Orth DN: Cushing's syndrome , in Krieger DT, Bardin CW (eds): Current Therapy in Endocrinology 1983-1984 . Burlington, Ontario, B. C. Decker, 1983, pp 119-127. 23. Pont A, Gutierrez-Hartman A: Cushing's disease: Recurrence after a surgically induced remission. Arch Intern Med 1979;139:938-940.Crossref 24. Ludecke D, Kautzky R, Saeger W, et al: Selective removal of hypersecreting pituitary adenomas. Acta Neurochir 1976;35:27-42.Crossref 25. Strott CA, Nugent CA, Tyler FH: Cushing's syndrome caused by bronchial adenomas. Am J Med 1968;44:97-103.Crossref 26. Aron DC, Findling JW, Fitzgerald PA, et al: Cushing's syndrome: Problems in management. Endocr Rev 1982;3:229-244.Crossref 27. Doppman JL, Oldfield E, Krudy AG, et al: Petrosal sinus sampling for Cushing syndrome: Anatomical and technical considerations. Radiology 1984;150:99-103.Crossref 28. Prinz RA, Brooks MH, Lawrence AM, et al: The continued importance of adrenalectomy in the treatment of Cushing's disease. Arch Surg 1979;114:481-484.Crossref 29. Delaney JP, Solomkim JS, Jacabson ME, et al: Surgical management of Cushing's syndrome. Surgery 1978;84:465-470. 30. Nelson DH, Meakin JW, Thorn GW: ACTH-producing pituitary tumors following adrenalectomy for Cushing's syndrome. Ann Intern Med 1960;52:560-569.Crossref 31. Moore TJ, Dluhy RG, Williams GH, et al: Nelson's syndrome: Frequency, prognosis, and effect of prior irradiation. Ann Intern Med 1976;85: 731-734.Crossref 32. Jennings AS, Liddle GW, Orth DN: Results of treating childhood Cushing's disease with pituitary irradiation. N Engl J Med 1977;297:957-962.Crossref 33. Orth DN, Liddle GW: Results of treatment in 108 patients with Cushing's syndrome. N Engl J Med 1971;285:243-247.Crossref 34. Barnett AH, Livesey JH, Friday K, et al: Comparison of preoperative and postoperative ACTH concentrations after bilateral adrenalectomy in Cushing's disease. Clin Endocrinol 1983;18:301-305.Crossref 35. Styne DM, Grumbach MM, Kaplan SL, et al: Treatment of Cushing's disease in childhood and adolescence by transsphenoidal microadenomectomy. N Engl J Med 1984;310:889-893.Crossref 36. McArthur RG, Hayles AB, Salassa RM: Childhood Cushing disease: Results of bilateral adrenalectomy. J Pediatr 1979;95:214-219.Crossref 37. Krieger DT, Amorosa L, Linick F: Cyproheptadine-induced remission of Cushing's disease. N Engl J Med 1975;293:893-896.Crossref 38. Krieger DT: Cyproheptadine: Drug therapy for Cushing's disease , in Muller EE (ed): Neuroactive Drugs in Endocrinology . Amsterdam, Elsevier-North Holland, 1980, pp 361-370. 39. Aronin N, Krieger DT: Sustained remission of Nelson's syndrome after stopping cyproheptadine treatment. N Engl J Med 1980;302:453-455.Crossref 40. Hsu T-H, Gann DS, Tasn K-W, et al: Cyproheptadine in the control of Cushing's disease. Johns Hopkins Med J 1981;149:77-83. 41. Lamberts SWJ, de Lange SA, Stefanko SZ: Adrenocorticotropinsecreting pituitary adenomas originate from the anterior or the intermediate lobe in Cushing's disease: Differences in the regulation of hormone secretion. J Clin Endocrinol Metabol 1982;54:286-291.Crossref 42. Miura K, Aida M, Mihara A, et al: Treatment of Cushing's disease with reserpine and pituitary irradiation. J Clin Endocrinol Metabol 1975;41:511-526.Crossref 43. Dornhorst A, Jenkins JS, Lamberts SWJ, et al; The evaluation of sodium valproate in the treatment of Nelson's syndrome. J Clin Endocrinol Metabol 1983;56:985-991.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Jun 1, 1985

References