This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsPreview
Unable to display preview. Download preview PDF.
References
Ajani JA, Legha SS, Karlin DA, Stratton Hill C (1983) Combination chemotherapy of metastatic carcinoid tumors with 5-FU, adriamycin, mitomycin and methyl-CCNU. Proc Am Assoc Clin Oncol 2:124 (Abstract C-486)
Amikura K, Alexander HR, Norton JA, Doppman JL, Jensen RT, Nieman L, Cutler G, Chrousos G, Fraker DL (1995) Role of surgery in management of adrenocorticotropic hormone-producing islet cell tumors of the pancreas. Surgery 118:1125–1130
Aniszewski JP, Young WF Jr, Thompson GB, Grant CS, van Heerden JA (2001) Cushing syndrome due to ectopic adrenocorticotropic hormone secretion. World J Surg 25:934–940
Arioglu E, Doppman J, Gomes M, Kleiner D, Mauro D, Barlow C, Papanicolaou DA (1998) Cushing’s syndrome caused by corticotropin secretion by pulmonary tumor-lets. N Engl J Med 24;339:883–886
Arnaldi G, de Keyzer Y, Gasc JM, Clauser E, Bertagna X (1998) Vasopressin receptors modulate the pharmacological phenotypes of Cushing’s syndrome. Endocr Res 24: 807–816
Aron DC, Raff H, Findling JW (1997) Effectiveness versus efficacy: the limited value in clinical practice of high dose dexamethasone suppression testing in the differential diagnosis of adrenocorticotropin-dependent Cushing’s syndrome. J Clin Endocrinol Metab 82:1780–1785
Bertagna X (1994) Proopiomelanocortin-derived peptides. Endocrinol Metab Clin North Am 23:467–485
Beuschlein F, Hammer GD (2002) Ectopic proopiomelanocortin syndrome. Endocrinol Metab Clin North Am 31:191–234
Blumgart LH, Allison DJ (1982) Resection and embolization in the management of secondary hepatic tumors. World J Surg 6:32–45
Bukowski RM, John KG, Peterson RF, Stephens RL, Rivkin SE, Neilan B, Costanzi JH (1987) A phase II trial of combination chemotherapy in patients with metastatic carcinoid tumors. A Southwest Oncology Group Study. Cancer 60:2891–2895
Christin-Maitre S, Chabbert-Buffet N, Mure A, Boukhris R, Bouchard P (1996) Use of somatostatin analog for localization and treatment of ACTH secreting bronchial carcinoid tumor. Chest 109:845–846
Clark ES, Carney JA (1984) Pancreatic islet cell tumor associated with Cushing’s syndrome. Am J Surg Pathol 8:917–924
Comi RJ, Gorden P (1998) Long-term medical treatment of ectopic ACTH syndrome. South Med J 91:1014–1018
De Keyzer Y, Bertagna X, Lenne F, Girard F, Luton JP, Kahn A (1985) Altered proopiomelanocortin gene expression in adrenocorticotropin-producing nonpituitary tumors. Comparative studies with corticotropic adenomas and normal pituitaries. J Clin Invest 76:1892–1898
De Keyzer Y, Bertagna X, Luton JP, Kahn A (1989) Variable modes of proopiomelanocortin gene transcription in human tumors. Mol Endocrinol 3:215–223
De Keyzer Y, Lenne F, Auzan C, Jegou S, Rene P, Vaudry H, Kuhn JM, Luton JP, Clauser E, Bertagna X (1996) The pituitary V3 vasopressin receptor and the corticotroph phenotype in ectopic ACTH syndrome. J Clin Invest 97:1311–1318
De Keyzer Y, Raffin-Sanson ML, Picon A, Bertagna X (2001) Syndromes of ectopic ACTH secretion: recent pathophysiological aspects. In: Margioris AN, Chrousos GP (eds) Adrenal disorders. Humana Press, Totowa, NJ, pp 165–178
DeBold CR, Menefee JK, Nicholson WE, Orth DN (1988) Proopiomelanocortin gene is expressed in many normal human tissues and in tumors not associated with ectopic adrenocorticotropin syndrome. Mol Endocrinol 2:862–870
Delisle L, Boyer MJ, Warr D, Killinger D, Payne D, Yeoh JL, Feld R (1993) Ectopic corticotropin syndrome and small-cell carcinoma of the lung. Clinical features, outcome, and complications. Ann Intern Med 153:746–752
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
Doppman JL, Chang R, Oldfield EH, Chrousos G, Stratakis CA, Nieman LK (1999) The hypoplastic inferior petrosal sinus: a potential source of false-negative results in petrosal sampling for Cushing’s disease. J Clin Endocrinol Metab 84:533–540
Doppman JL, Nieman LK, Cutler GB Jr, Chrousos GP, Fraker DL, Norton JA, Jensen RT (1994) Adrenocorticotropic hormone-secreting islet cell tumors: are they always malignant? Radiology 190:59–64
Doppman JL, Pass HI, Nieman LK, Findling JW, Dwyer AJ, Feuerstein IM, Ling A, Travis WD, Cutler GB Jr, Chrousos GP, Loriaux DL (1991) Detection of ACTH-producing bronchial carcinoid tumors: MR imaging vs CT. AJR 156:39–43
Doppman JL (1994) Somatostatin receptor scintigraphy and the ectopic ACTH syndrome. The solution or just another test? Am J Med 96:303–304
Du Pont AG, Somers G, Van Steiterghem AC, Warson F, Vanhaelst L (1984) Ectopic adrenocorticotropin production: disappearance after removal of inflammatory tissue. J Clin Endocrinol Metab 58:654–658
Eriksson B, Oberg K, Alm G, Karlsson A, Lundqvist G, Andersson T, Wilander E, Wide L (1986) Treatment of malignant endocrine pancreatic tumors with human leucocyte interferon. Lancet 2:1307–1309
Farwell AP, Devlin JT, Stewart JA (1988) Total suppression of cortisol excretion by ketoconazole in the therapy of the ectopic adrenocorticotropic hormone syndrome. Am J Med 84:1063–1066
Findling JW, Kehoe ME, Shaker JL, Raff H (1991) Routine inferior petrosal sinus sampling in the differential diagnosis of adrenocortical (ACTH)-dependent Cushing’s syndrome: early recognition of the occult ectopic ACTH syndrome. J Clin Endocrinol Metab 73:408–413
Friedman TC, Loh YP, Birch NP (1994) In vitro processing of proopiomelanocortin by recombinant PC1 (SPC3). Endocrinology 135:854–862
Grossman AB, Howlett TA, Perry L, Coy DH, Savage MO, Lavender-P, Rees LH, Besser GM (1988) CRF in the differential diagnosis of Cushing’s syndrome: a comparison with the dexamethasone suppression test. Clin Endocrinol (Oxf) 29:167–178
Harbour JW, Lai SL, Whang-Peng J, Gazdar AF, Minna JD, Kaye FJ (1988) Abnormalities in structure and expression of the human retinoblastoma gene in SCLC. Science 241:353–357
Howlett TA, Drury PL, Perry L, Doniach I, Rees LH, Besser GM (1986) Diagnosis and management of ACTH-dependent Cushing’s syndrome: comparison of the features in ectopic and pituitary ACTH production. Clin Endocrinol (Oxf) 24:699–713
Invitti C, Giraldi FP, Cavagnini F (1999) Inferior petrosal sinus sampling in patients with Cushing’s syndrome and contradictory responses to dynamic testing. Clin Endocrinol (Oxf) 51:255–257
Invitti C, Giraldi FP, de Martin M, Cavagnini F (1999) Diagnosis and management of Cushing’s syndrome: results of an Italian multicentre study. Study Group of the Italian Society of Endocrinology on the Pathophysiology of the Hypothalamic-Pituitary-Adrenal Axis. J Clin Endocrinol Metab 84:440–448
Ishido H, Yamashita N, Kitaoka M, Tanaka Y, Ogata E (1994) A case of ectopic ACTH syndrome associated with Zollinger-Ellison syndrome: long-term survival with chemical adrenalectomy. Endocr J 41:171–176
Jeannotte L, Burbach JP, Drouin J (1987) Unusual proopiomelanocortin ribonucleic acids in extrapituitary tissues: intronless transcripts in testes and log poly(A) tails in hypothalamus. Mol Endocr 1:749–757
Kaltsas T, Krassas GE, Pontikides N, Tsotsia E, Tsagarakis S (2003) Cyclical Cushing syndrome caused by intermittent ectopic ACTH hormonogenesis responsive to somatostatin analogues. Endocrinologist 13:7–11
Kato Y, Ferguson TB, Bennett DE, Burford TH (1969) Oat cell carcinoma of the lung. A review of 138 cases. Cancer 23:517–524
Kaye TB, Crapo L (1990) The Cushing syndrome: An update on diagnostic tests. Ann Intern Med 112:434–444
Kuhn JM, Proeschel MF, Seurin DJ, Bertagna XY, Luton JP, Girard FL (1989) Comparative assessment of ACTH and lipotropin plasma levels in the diagnosis and follow-up of patients with Cushing’s syndrome: a study of 210 cases. Am J Med 86:678–684
Kulke MH, Mayer RJ (1999) Carcinoid tumors. N Engl J Med 340:858–868
Lam EW, La Thangue NB (1994) DP and E2F proteins: coordinating transcription with cell cycle progression. Curr Opin Cell Biol 6:859–866. Review
Lamberts SWS, Krenning EP, Reubi JC (1991) The role of somatostatin and its analogue in the diagnosis and treatment of tumors. Endocr Rev 12:450–482
Liddle GW, Island DP, Meador CK (1962) Normal and abnormal regulation of corticotropin secretion in man. Recent Prog Horm Res 18:125–166
Lyons DF, Eisen BR, Clark MR, Pysher TJ, Welsh JD, Kem DC (1984) Concurrent Cushing’s and Zollinger-Ellison syndromes in a patient with islet cell carcinoma. Case report and review of the literature. Am J Med 76: 729–733
Makita M, Maeda Y, Hashimoto K, Nakase K, Takenaka K, Shinagawa K, Ishimaru F, Ikeda K, Niiya K, Ogura T, Harada M (2003) Acute lymphoblastic leukemia with large molecular ACTH production. Ann Hematol 82: 448–451
Malerbi DA, Mendonca BB, Liberman B, Toledo SP, Corradini MC, Cunha-Neto MB, Fragoso MC, Wajchenberg BL (1993) The desmopressin stimulation test in the differential diagnosis of Cushing’s syndrome. Clin Endocrinol (Oxf) 38:463–472
Miller JW, Crapo L (1993) The medical treatment of Cushing’s syndrome. Endocr Rev 14:443–458
Moertel CG, Kvols LK, O’Connell MJ, Rubin J (1991) Treatment of neuroendocrine carcinomas with combined etoposide and cisplatin: evidence of major therapeutic activity in the anaplastic variants of these neoplasms. Cancer 68:227–232
Naidich DP (1990) CT/MR correlation in the evaluation of tracheobronchial neoplasia. Radiol Clin North Am 28: 555–571
Newell-Price J, Perry L, Medbak S, Monson J, Savage M, Besser M, Grossman A (1997) A combined test using desmopressin and corticotropin-releasing hormone in the differential diagnosis of Cushing’s syndrome. J Clin Endocrinol Metab 82:176–181
Newell-Price J, Trainer P, Besser M, Grossman A (1998) The diagnosis and differential diagnosis of Cushing’s syndrome and pseudo-Cushing’s states. Endocr Rev 19: 647–672
Nieman LK, Chrousos GP, Oldfield EH, Avgerinos PC, Cutler GB Jr, Loriaux DL (1986) The ovine corticotropin-releasing hormone stimulation test and the dexamethasone suppression test in the differential diagnosis of Cushing’s syndrome. Ann Intern Med 105:862–867
Oberg K, Norheim I, Alm G (1989) Treatment of malignant carcinoid tumors: a randomized controlled study of streptozotocin plus 5-FU and human leucocyte interferon. Eur J Cancer Clin Oncol 25:1475–1479
Odell WD (1991) Ectopic ACTH secretion: a misnomer. Endocrinol Metab Clin North Am 20:371–379
Oldfield EH, Doppman JL, Nieman LK, Chrousos GP, Miller DL, Ktaz DA, Cutler 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 325:896–905
Orth DN (1995) Cushing’s syndrome. N Engl J Med 332:791–803
Phlipponneau M, Nocaudie M, Epelbaum J, De Keyzer Y, Lalau JD, Marchandise X, Bertagna X (1994) Somatostatin analogs for the localization and preoperative treatment of an adrenocorticotropin-secreting bronchial carcinoid tumor. J Clin Endocrinol Metab 78:20–24
Picon A, Bertagna X, de Keyzer Y (1999) Analysis of the human proopiomelanocortin gene promoter in a small cell lung carcinoma cell line reveals an unusual role for E2F transcription factors. Oncogene 18:2627–2633
Raffin-Sanson ML, Massias JF, Dumont C, Raux-Demay MC, Proeschel MF, Luton JP, Bertagna X (1996) High plasma proopiomelanocortin in aggressive adrenocorticotropin-secreting tumors. J Clin Endocrinol Metab 81: 4272–4277
Raffin-Sanson ML, de Keyzer Y, Bertagna X (2003) Proopiomelanocortin, a polypeptide precursor with multiple functions: from physiology to pathological conditions. Eur J Endocrinol 149:79–90
Rena O, Filosso PL, Maggi G, Casadio C (2003) Neuroendocrine tumors (carcinoid) of the thymic gland. Ann Thorac Surg 75:633
Seidah NG, Marcinkiewicz M, Benjannet S, Gaspar L, Beaubien G, Mattei MG et al. (1991) Cloning and primary sequence of a mouse candidate prohormone convertase PC1 homologous to PC2, furin, and Kex2: distinct chromosomal localization and messenger RNA distribution in brain and pituitary compared to PC2. Mol Endocrinol 5:111–122
Shepherd FA, Laskey J, Evans WK, et al. (1992) Cushing’s syndrome associated with ectopic corticotropin production and small-cell lung cancer. J Clin Oncol 10:21–27
Shrager JB, Wright CD, Wain JC, Torchiana DF, Grillo HC, Mathisen DJ (1997) Bronchopulmonary carcinoid tumors associated with Cushing’s syndrome: a more aggressive type of typical carcinoids. J Thorac Cardiovasc Surg 114:367–375
Smeekens SP, Steiner DF (1990) Identification of a human insulinoma cDNA encoding a novel mammalian protein structurally related to the yeast dibasic processing protease Kex 2. J Biol Chem 265:2997–3000
Tabarin A, Valli N, Chanson P, Bachelot Y, Rohmer V, Bex-Bachellerie B, Catargi B, Roger P, Laurent F (1999) Usefulness of somatostatin receptor scintigraphy in patients with occult ectopic adrenocorticotropin syndrome. J Clin Endocrinol Metab 84:1193–1202
Takahashi H, Hakamata Y, Watanabe Y, Kikuno R, Miyata T, Numa S (1983) Complete nucleotide sequence of the human corticotropin-beta-lipotropin precursor gene. Nucleic Acids Res 11:6847–6858
Terzolo M, Reimondo G, Ali A, Borretta G, Cesario F, Pia A, Paccotti P, Angeli A (2001) The limited value of the desmopressin test in the diagnostic approach to Cushing’s syndrome. Clin Endocrinol (Oxf) 54:609–616
Terzolo M, Reimondo G, Ali A, Bovio S, Daffara F, Paccotti P, Angeli A (2001) Ectopic ACTH syndrome: molecular bases and clinical heterogeneity. Ann Oncol 12Suppl 2:S83–87
Texier PL, De Keyzer Y, Lacave R, Vieau D, Lenne F, Rojas-Miranda A, Verley JM, Luton JP, Kahn A, Bertagna X (1991) Proopiomelanocortin gene expression in normal and tumoral human lung. J Clin Endocrinol Metab 73:414–420
Torpy DJ, Chen CC, Mullen N, Doppman JL, Carrasquillo JA, Chrousos GP, Nieman LK (1999) Lack of utility of 111In-Pentetreotide scintigraphy in localizing ectopic ACTH producing tumors: follow-up of 18 patients. J Clin Endocrinol Metab 84:1186–1192
Trainer PJ, Grossman A (1991) The diagnosis and differential diagnosis of Cushing’s syndrome. Clin Endocrinol (Oxf) 34:317–330
Tsagarakis S, Christoforaki M, Giannopoulou H, Rondogianni F, Housianakou I, Malagari C, Rontogianni D, Bellenis I, Thalassinos N (2003) A reappraisal of the utility of somatostatin receptor scintigraphy in patients with ectopic adrenocorticotropin Cushing’s syndrome. J Clin Endocrinol Metab 88:4754–4758
Tsagarakis S, Giannakenas C, Vassilakos PJ, Platis O, Belenis I, Kaskarelis J, Rontoyianni D, Thalassinos N (1995) Successful localization of an occult ACTH-secreting bronchial carcinoid tumour with 111 indium-DTPA labelled octreotide. Clin Endocrinol (Oxf). 43:763–767
Tsagarakis S, Kaskarelis IS, Kokkoris P, Malagari C, Thalassinos N (2000) The application of combined stimulation with CRH and desmopressin during bilateral petrosal sinus sampling in patients with Cushing’s syndrome. Clin Endocrinol (Oxf) 52:355–361
Tsagarakis S, Thalassinos N (2001) The desmopressin test: a new tool in the functional evaluation of the pituitary-adrenal axis in patients with Cushing’s syndrome? In: Margioris AN, Chrousos GP (eds) Adrenal disorders. Humana Press, Totowa, NJ, pp 193–202
Tsagarakis S, Tsigos C, Vasiliou V, Tsiotra P, Kaskarelis J, Sotiropoulou C, Raptis SA, Thalassinos N (2002) The desmopressin and combined CRH-desmopressin tests in the differential diagnosis of ACTH-dependent Cushing’s syndrome: constraints imposed by the expression of V2 vasopressin receptors in tumors with ectopic ACTH secretion. J Clin Endocrinol Metab 87:1646–1653
Vaidya B, Richardson D, Hilton CJ, Kendall-Taylor P (1997) Adrenocorticotropin-secreting carcinoid tumour identified and treated 12 years after presentation with Cushing’s syndrome. Postgrad Med J 73:737–739
Van Coevorden A, Laurent E, Rickaert F, van Reeth O, Van Cauter E, Mockel J (1990) Cushing’s syndrome with intermittent ectopic ACTH production. J Endocrinol Invest 13:317–326
Van den Bruel A, Bex M, Van Dorpe J, Heyns W, Bouillon R (1998) Occult ectopic ACTH secretion due to recurrent lung carcinoid: long-term control of hypercortisolism by continuous subcutaneous infusion of octreotide. Clin Endocrinol (Oxf) 49:541–546
Vella A, Thompson GB, Grant CS, van Heerden JA, Farley DR, Young WF Jr (2001) Laparoscopic adrenalectomy for adrenocorticotropin-dependent Cushing’s syndrome. J Clin Endocrinol Metab 86:1596–1599
Vieau D, Seidah NG, Mbikay M, Chretien M, Bertagna X (1994) Expression of the prohormone convertase PC2 correlates with the presence of corticotropin-like intermediate lobe peptide in human adrenocorticotropin-secreting tumors. J Clin Endocrinol Metab 79:1503–1506
Vignati F, Loli P (1996) Additive effect of ketoconazole and octreotide in the treatment of severe adrenocorticotropin-dependent hypercortisolism. J Clin Endocrinol Metab 81:2885–2890
Vincent JM, Trainer PJ, Reznek RH, Marcus AJ, Dacie JE, Armstrong P, Besser GM (1993) The radiological investigation of occult ectopic ACTH-dependent Cushing’s syndrome. Clin Radiol 48:11–17
Wajchenberg BL, Mendonca BB, Liberman B, Pereira MA, Carneiro PC, Wakamatsu A, Kirschner MA (1994) Ectopic adrenocorticotropic hormone syndrome. Endocr Rev 15:752–787
Weiss M, Yellin A, Husza’r M, Eisenstein Z, Bar-Ziv J, Krausz Y (1994) Localization of adrenocorticotropic hormone-secreting bronchial carcinoid tumor by somatostatin-receptor scintigraphy. Ann Intern Med 121: 198–199
White A, Clark AJL (1993) The cellular and molecular basis of the ectopic ACTH syndrome. Clin Endocrinol (Oxf) 39:131–141
White A, Gibson S (1998) ACTH precursors: biological significance and clinical relevance. Clin Endocrinol (Oxf) 48:251–255
Winocour PH, Tong P, White J, Edwards A, White A, Gibson GJ, Clark F (1993) Unusual presentation and course of Cushing’s syndrome due to ectopic ACTH secretion by a bronchial carcinoma. Respir Med 87:149–151
Zeiger MA, Pass HI, Doppman JD, Nieman LK, Chrousos GP, Cutler GB Jr, Jensen RT, Norton JA (1992) Surgical strategy in the management of non-small cell ectopic adrenocorticotropic hormone syndrome. Surgery 112: 994–1000
Zhou A, Bloomquist BT, Mains RE (1993) The prohormone convertases PC1 and PC2 mediate distinct endoproteolytic cleavages in a strict temporal order during proopiomelanocortin biosynthetic processing. J Biol Chem 268:1763–1769
Zhu L, Domenico DR, Howard JM (1996) Metastatic pancreatic neuroendocrine carcinoma causing Cushing’s syndrome. ACTH secretion by metastases 3 years after resection of nonfunctioning primary cancer. Int J Pancreatol 19:205–208
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2005 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Vassiliadi, D., Tsagarakis, S., Thalassinos, N. (2005). Ectopic Cushing’s Syndrome. In: Linos, D., van Heerden, J.A. (eds) Adrenal Glands. Springer, Berlin, Heidelberg. https://doi.org/10.1007/3-540-26861-8_8
Download citation
DOI: https://doi.org/10.1007/3-540-26861-8_8
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-41099-7
Online ISBN: 978-3-540-26861-1
eBook Packages: MedicineMedicine (R0)