Ectopic adrenocorticotropin syndrome: Diagnosis and treatment

Current Opinion in Endocrinology and Diabetes

Volumn 13, Issue 3, 2006, Pages 237-241

  Ray, David W ^a  

^a UNIVERSITY OF MANCHESTER   (United Kingdom)

Author keywords

Adrenocorticotropin; Carcinoid; Ectopic; POMC gene

Indexed keywords

DNA; DOPAMINE RECEPTOR STIMULATING AGENT; ETOMIDATE; KETOCONAZOLE; METYRAPONE; MITOTANE; SOMATOSTATIN DERIVATIVE; TRANSCRIPTION FACTOR;

ADRENALECTOMY; BLOOD SAMPLING; CLINICAL FEATURE; COMPUTER ASSISTED TOMOGRAPHY; CUSHING DISEASE; DEXAMETHASONE SUPPRESSION TEST; ECTOPIC CORTICOTROPIN PRODUCTION; GENE; GENE CONTROL; GENE EXPRESSION; HUMAN; HYPOPHYSIS; NUCLEAR MAGNETIC RESONANCE IMAGING; OBESITY; POMC GENE; PROMOTER REGION; PROTEIN ASSEMBLY; RADIOGRAPHY; REVIEW; SCINTISCANNING; UNITED KINGDOM; UNITED STATES;

EID: 33646687732     PISSN: 10683097     EISSN: None     Source Type: Journal    
DOI: 10.1097/01.med.0000224802.85953.37     Document Type: Review

References (32)

1. White A, Clark AJ. The cellular and molecular basis of the ectopic ACTH syndrome. Clin Endocrinol (Oxford) 1993; 39:131-141.
2. Delisle L, Boyer MJ, Warr D, et al. Ectopic corticotropin syndrome and small-cell carcinoma of the lung: clinical features, outcome, and complications. Arch Intern Med 1993; 153:746-752.
3. White A, Clark AJ, Stewart MF. The synthesis of ACTH and related peptides by tumours. Baillieres Clin Endocrinol Metab 1990; 4:1-27.
4. Newell-Price J. Proopiomelanocortin gene expression and DNA methylation: implications for Cushing's syndrome and beyond. J Endocrinol 2003; 177:365-372.
5. DeBold CR, Menefee JK, Nicholson WE, Orth DN. Proopiomelanocortin gene is expressed in many normal human tissues and in tumors not associated with ectopic adrenocorticotropin syndrome. Mol Endocrinol 1988; 2:862-870.
6. DeBold CR, Nicholson WE, Orth DN. Immunoreactive proopiomelanocortin (POMC) peptides and POMC-like messenger ribonucleic acid are present in many rat nonpituitary tissues. Endocrinology 1988; 122:2648-2657.
7. Ye L, Li X, Kong X, et al. Hypomethylation in the promoter region of POMC gene correlates with ectopic overexpression in thymic carcinoids. J Endocrinol 2005; 185:337-343.
8. Newell-Price J, King P, Clark AJ. The CpG island promoter of the human proopiomelanocortin gene is methylated in nonexpressing normal tissue and tumors and represses expression. Mol Endocrinol 2001; 15:338-348.
9. Clark AJ, Lavender PM, Coates P, et al. In vitro and in vivo analysis of the processing and fate of the peptide products of the short proopiomelanocortin mRNA. Mol Endocrinol 1990; 4:1737-1743.
10. Clark AJ, Lavender PM, Besser GM, Rees LH. Pro-opiomelanocortin mRNA size heterogeneity in ACTH-dependent Cushing's syndrome. J Mol Endocrinol 1989; 2:3-9.
11. Stewart MF, Crosby SR, Gibson S, et al. Small cell lung cancer cell lines secrete predominantly ACTH precursor peptides not ACTH. Br J Cancer 1989; 60:20-24.
12. Stewart PM, Gibson S, Crosby SR, et al. ACTH precursors characterize the ectopic ACTH syndrome. Clin Endocrinol (Oxford) 1994; 40:199-204.
13. Bertagna XY, Nicholson WE, Pettengill OS, et al. Ectopic production of high molecular weight calcitonin and corticotropin by human small cell carcinoma cells in tissue culture: evidence for separate precursors. J Clin Endocrinol Metab 1978; 47:1390-1393.
14. Ray DW, Gibson S, Crosby SR, et al. Elevated levels of adrenocorticotropin (ACTH) precursors in post-adrenalectomy Cushing's disease and their regulation by glucocorticoids. J Clin Endocrinol Metab 1995; 80:2430-2436.
15. Bicknell AB, Lomthaisong K, Woods RJ, et al. Characterization of a serine protease that cleaves pro-gamma-melanotropin at the adrenal to stimulate growth. Cell 2001; 105:903-912.
16. Wajchenberg BL, Mendonca BB, Liberman B, et al. Ectopic adrenocorticotropic hormone syndrome. Endocr Rev 1994; 15:752-787.
17. Ilias I, Torpy DJ, Pacak K, et al. Cushing's syndrome due to ectopic corticotropin secretion: twenty years' experience at the National Institutes of Health. J Clin Endocrinol Metab 2005; 90:4955-4962. Very useful and important series of 90 patients with ectopic ACTH syndrome. Good summary of investigation pitfalls, and outcome data from intervention.
18. Findling JW, Tyrrell JB. Occult ectopic secretion of corticotropin. Arch Intern Med 1986; 146:929-933.
19. Isidori AM, Kaltsas GA, Pozza C, et al. The ectopic ACTH syndrome: clinical features, diagnosis, management and long-term follow-up. J Clin Endocrinol Metab 2006; 91:371-377. Very useful, long-term outcome study from a single UK referral centre. All patients were investigated and managed to protocol, and outcome data are clearly presented.
20. Torpy DJ, Mullen N, Ilias I, Nieman LK. Association of hypertension and hypokalemia with Cushing's syndrome caused by ectopic ACTH secretion: a series of 58 cases. Ann NY Acad Sci 2002; 970:134-144.
21. Findling JW, Aron DC, Tyrrell JB, et al. Selective venous sampling for ACTH in Cushing's syndrome: differentiation between Cushing disease and the ectopic ACTH syndrome. Ann Intern Med 1981; 94:647-652.
22. Doppman JL, Nieman LK, Chang R, et al. Selective venous sampling from the cavernous sinuses is not a more reliable technique than sampling from the inferior petrosal sinuses in Cushing's syndrome. J Clin Endocrinol Metab 1995; 80:2485-2489.
23. Oldfield EH, Chrousos GP, Schulte HM, et al. Preoperative lateralization of ACTH-secreting pituitary microadenomas by bilateral and simultaneous inferior petrosal venous sinus sampling. N Engl J Med 1985; 312:100-103.
24. Findling JW, Raff H. Diagnosis and differential diagnosis of Cushing's syndrome. Endocrinol Metab Clin North Am 2001; 30:729-747.
25. Ilias I, Chang R, Pacak K, et al. Jugular venous sampling: an alternative to petrosal sinus sampling for the diagnostic evaluation of adrenocorticotropic hormone-dependent Cushing's syndrome. J Clin Endocrinol Metab 2004; 89:3795-3800. Useful paper identifying the risks of petrosal sinus sampling, and the possible use of a simpler, presumably safer, jugular venous sampling approach.
26. Findling JW, Kehoe ME, Raff H. Identification of patients with Cushing's disease with negative pituitary adrenocorticotropin gradients during inferior petrosal sinus sampling: prolactin as an index of pituitary venous effluent. J Clin Endocrinol Metab 2004; 89:6005-6009. Useful paper that indicates potential ways to improve the diagnostic performace of petrosal sinus sampling by correcting for prolactin in effluent blood.
27. 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; 89:2214-2221. Useful paper summarizing the state of the art with scintigraphy in diagnosis of ectopic ACTH syndrome causing tumors.
28. Tsagarakis S, Christoforaki M, Giannopoulou H, et al. A reappraisal of the utility of somatostatin receptor scintigraphy in patients with ectopic adrenocorticotropin Cushing's syndrome. J Clin Endocrinol Metab 2003; 88:4754-4758.
29. Farrell WE, Clark AJ, Stewart MF, et al. Bromocriptine inhibits pro-opiomelanocortin mRNA and ACTH precursor secretion in small cell lung cancer cell lines. J Clin Invest 1992; 90:705-710.
30. Pivonello R, Ferone D, Lamberts SW, Colao A. Cabergoline plus lanreotide for ectopic Cushing's syndrome. N Engl J Med 2005; 352:2457-2458. Thought-provoking case report of the use of lanreotide combined with cabergoline in treatment of a patient with ectopic ACTH syndrome.
31. de Herder WW, Lamberts SW. Is there a role for somatostatin and its analogs in Cushing's syndrome? Metabolism 1996; 45:83-85.
32. de Herder WW, van der Lely AJ, Lamberts SW. Somatostatin analogue treatment of neuroendocrine tumours. Postgrad Med J 1996; 72:403-408.