ectopic acth syndrome


Summary: Symptom complex due to ACTH production by non-pituitary neoplasms.

Top Publications

  1. Kanno K, Morokuma Y, Tateno T, Hirono Y, Taki K, Osamura R, et al. Olfactory neuroblastoma causing ectopic ACTH syndrome. Endocr J. 2005;52:675-81 pubmed
    ..Thus, this is the second rare case with ectopic ACTH syndrome caused by olfactory neuroblastoma thus far reported.
  2. Wang W, Ye L, Bi Y, Zhao H, Sun S, Tang Z, et al. Six cases of ectopic ACTH syndrome caused by thymic carcinoid. J Endocrinol Invest. 2006;29:293-7 pubmed
    The ectopic ACTH syndrome (EAS) caused by thymic carcinoid is rare and its diagnosis remains a challenge to the endocrinologist. Here are six cases of EAS with a typical Cushing habitus accompanied by hyperpigmentation and hypokalemia...
  3. Salgado L, Fragoso M, Knoepfelmacher M, Machado M, Domenice S, Pereira M, et al. Ectopic ACTH syndrome: our experience with 25 cases. Eur J Endocrinol. 2006;155:725-33 pubmed
    b>Ectopic ACTH syndrome (EAS) occurs in about 5-10% of all patients with ACTH-dependent hypercortisolism with most of them caused by intrathoracic neoplasms. It may be associated with overt malignancies or with occult and indolent tumors...
  4. Tani Y, Sugiyama T, Hirooka S, Izumiyama H, Hirata Y. Ectopic ACTH syndrome caused by bronchial carcinoid tumor indistinguishable from Cushing's disease. Endocr J. 2010;57:679-86 pubmed
    ..This was a diagnostic challenging case with ectopic ACTH syndrome indistinguishable from Cushing' s disease by various endocrine and imaging tests, among which SRS ..
  5. Isidori A, Lenzi A. Ectopic ACTH syndrome. Arq Bras Endocrinol Metabol. 2007;51:1217-25 pubmed
    ..Their prevalence and the best localization modalities are presented. Medical and surgical management is discussed on the basis of the extensive experience of major referral centres...
  6. de Keyzer Y, Lenne F, Auzan C, Jegou S, Rene P, Vaudry H, et al. The pituitary V3 vasopressin receptor and the corticotroph phenotype in ectopic ACTH syndrome. J Clin Invest. 1996;97:1311-8 pubmed
    ..Six of eight bronchial carcinoids responsible for the ectopic ACTH syndrome had both POMC and V3 receptor signals as high as those in ACTH-secreting pituitary adenomas; in contrast, ..
  7. Mintzer D, Zheng S, Nagamine M, Newman J, Benito M. Esthesioneuroblastoma (Olfactory Neuroblastoma) with Ectopic ACTH Syndrome: a multidisciplinary case presentation from the Joan Karnell cancer center of Pennsylvania Hospital. Oncologist. 2010;15:51-8 pubmed publisher
    ..The case of a patient with recurrent esthesioneuroblastoma complicated by ectopic adrenocorticotropic hormone production is presented, including the workup and management of this uncommon complication of an uncommon disease...
  8. Otsuka F, Miyoshi T, Murakami K, Inagaki K, Takeda M, Ujike K, et al. An extra-adrenal abdominal pheochromocytoma causing ectopic ACTH syndrome. Am J Hypertens. 2005;18:1364-8 pubmed
    ..This is the first report of ectopic ACTH syndrome induced by an extra-adrenal abdominal paraganglioma.
  9. Terzolo M, Reimondo G, Ali A, Bovio S, Daffara F, Paccotti P, et al. Ectopic ACTH syndrome: molecular bases and clinical heterogeneity. Ann Oncol. 2001;12 Suppl 2:S83-7 pubmed
    There are roughly two types of ectopic ACTH syndrome (EAS). one associated with overt malignancies and one with occult neoplasms...

More Information


  1. Salameh J, Borman K, Varkarakis G. Laparoscopic bilateral adrenalectomy for occult ectopic ACTH syndrome. J Laparoendosc Adv Surg Tech A. 2008;18:52-5 pubmed publisher
    ..Bilateral adrenalectomy is the most effective treatment for ectopic ACTH syndrome due to occult or disseminated tumors, but the open approach carries substantial morbidity...
  2. Kristiansen M, Rasmussen L, Olsen N, Asa S, Jørgensen J. Ectopic ACTH syndrome: discrepancy between somatostatin receptor status in vivo and ex vivo, and between immunostaining and gene transcription for POMC and CRH. Horm Res. 2002;57:200-4 pubmed
  3. Grunewald T, von Luettichau I, Welsch U, Dörr H, Höpner F, Kovacs K, et al. First report of ectopic ACTH syndrome and PTHrP-induced hypercalcemia due to a hepatoblastoma in a child. Eur J Endocrinol. 2010;162:813-8 pubmed publisher
    ..Only occasionally, endocrine-active tumors develop directly from hepatic tissue, and may lead to paraneoplastic syndromes (PNS). PNS mostly accompany malignancy of adulthood and are exceedingly rare in children...
  4. Jamieson L, Taylor S, Smith A, Bullock M, Davis M. Metastatic acinic cell carcinoma of the parotid gland with ectopic ACTH syndrome. Otolaryngol Head Neck Surg. 2007;136:149-50 pubmed
  5. Vilar L, Naves L, Freitas M, Moura E, Canadas V, Leal E, et al. [Endogenous Cushing's syndrome: clinical and laboratorial features in 73 cases]. Arq Bras Endocrinol Metabol. 2007;51:566-74 pubmed
    ..Among 10 patients that underwent bilateral inferior petrosal sinus sampling, a central-to-peripheral ACTH gradient > 3 after CRH or DDAVP had 90% sensitivity and 100% specificity for Cushing's disease. ..
  6. Reincke M, Allolio B, Deuss U, Winkelmann W. Gel chromatographic characterization of immunoreactive adrenocorticotropin in patients with ACTH hypersecretion. Klin Wochenschr. 1989;67:1096-100 pubmed
    ..4 patients with Addison's disease, 2 with Nelson's syndrome, 4 with Cushing's disease, 6 with the ectopic ACTH syndrome (2 bronchial carcinoma, 1 medullary carcinoma, 1 metastatic islett cell carcinoma, 1 benign bronchial ..
  7. Vassiliadi D, Tsagarakis S. Unusual causes of Cushing's syndrome. Arq Bras Endocrinol Metabol. 2007;51:1245-52 pubmed
    ..Such unusual presentations of CS illustrate why Cushing's syndrome represents one of the most puzzling endocrine syndromes. ..
  8. Bhatia P, Fung K, Edmonds M, Driedger A, Malthaner R. A case of bronchopulmonary carcinoid tumor: the role of octreotide scanning in localization of an ectopic source of ACTH. J Hosp Med. 2006;1:312-6 pubmed
    ..In situations when conventional imaging is not conclusive, octreotide scanning may be of help in determining the source of ectopic ACTH syndrome.
  9. Takahashi T, Hatao K, Yamashita Y, Tanizawa Y. Ectopic ACTH syndrome due to thymic atypical carcinoid treated with combination chemotherapy of cisplatin and etoposide. Intern Med. 2003;42:1197-201 pubmed
    A 21-year-old woman with Cushing's syndrome presented with a mediastinal tumor. Ectopic ACTH syndrome was diagnosed and the tumor was resected. The histopathological diagnosis was thymic atypical carcinoid...
  10. Korsic M, Plavsic V, Besenski N, Skoric T, Giljevic Z, Zarkovic K, et al. [Importance of the CRH (corticotropin releasing hormone) test in the differential diagnosis of Cushing's syndrome]. Lijec Vjesn. 2001;123:165-8 pubmed
    ..Among two patients with ectopic ACTH syndrome one had the significant increase of both hormones in CRH test...
  11. Zachäus M. [Paraneoplastic ectopic ACTH syndrome due to small cell lung cancer]. Internist (Berl). 2008;49:891; author reply 891-2 pubmed publisher
  12. Chiba H. [18-Hydroxycortisol, 18-oxocortisol, and 6beta-hydroxycortisol]. Nihon Rinsho. 2005;63 Suppl 8:311-5 pubmed
  13. Bondioni S, Mantovani G, Polentarutti N, Ambrosi B, Loli P, Peverelli E, et al. Evaluation of proopiomelanocortin mRNA in the peripheral blood from patients with Cushing's syndrome of different origin. J Endocrinol Invest. 2007;30:828-32 pubmed
    ..originating from a pituitary corticotroph adenoma (Cushing's disease) or from ectopic tumors (ectopic ACTH syndrome)...
  14. Reimondo G, Paccotti P, Minetto M, Termine A, Stura G, Bergui M, et al. The corticotrophin-releasing hormone test is the most reliable noninvasive method to differentiate pituitary from ectopic ACTH secretion in Cushing's syndrome. Clin Endocrinol (Oxf). 2003;58:718-24 pubmed
    ..Cushing's syndrome: 49 patients with proven Cushing's disease (CD) and 10 patients with proven ectopic ACTH syndrome (EAS)...
  15. Mayer S, Cypess A, Kocher O, Berman S, Huberman M, Hartzband P, et al. Uncommon presentations of some common malignancies: Case 1. Sequential paraneoplastic endocrine syndromes in small-cell lung cancer. J Clin Oncol. 2005;23:1312-4 pubmed
  16. Li Y, Peng Y, Jiang X, Cheng Y, Zhou W, Su T, et al. Whole exome sequencing of thymic neuroendocrine tumor with ectopic ACTH syndrome. Eur J Endocrinol. 2017;176:187-194 pubmed
    ..Somatic mutations of three potential tumor-related genes (HRAS, PAK1 and MEN1) might contribute to the tumorigenesis of thymic neuroendocrine tumors with EAS. ..
  17. Liu C, Lo J, Dowd C, Wilson C, Kunwar S, Aron D, et al. Cavernous and inferior petrosal sinus sampling in the evaluation of ACTH-dependent Cushing's syndrome. Clin Endocrinol (Oxf). 2004;61:478-86 pubmed
    ..were analysed, including 79 patients with suspected Cushing's disease (CD) and 16 patients with suspected ectopic ACTH syndrome (EAS)...
  18. Johnson T, Canada T. Etomidate use for Cushing's syndrome caused by an ectopic adrenocorticotropic hormone-producing tumor. Ann Pharmacother. 2007;41:350-3 pubmed
    ..There were no obvious problems with stability when undiluted etomidate 2 mg/mL was infused through a dedicated central venous catheter lumen. ..
  19. Thomson S, Pirie F. Every picture tells a story. S Afr Med J. 2002;92:701 pubmed
  20. Hou L, Harshbarger T, Herrick M, Tse V. Suprasellar adrenocorticotropic hormone-secreting ectopic pituitary adenoma: case report and literature Review. Neurosurgery. 2002;50:618-25 pubmed
    ..Ectopic pituitary adenomas should be considered in the differential diagnosis for all patients with Cushing's syndrome. Furthermore, surgical approaches should be chosen carefully once the diagnosis of ectopic pituitary adenoma is made. ..
  21. Barbetta L, Dall Asta C, Re T, Colombo P, Travaglini P, Ambrosi B. Androgen secretion in ectopic ACTH syndrome and in Cushing's disease: modifications before and after surgery. Horm Metab Res. 2001;33:596-601 pubmed
    ..It is suggested that in ACTH-secreting non-pituitary tumours, the production of a POMC-derived peptide, although unidentified, may lead to preferentially stimulated androstenedione secretion, without affecting other enzymatic pathways. ..
  22. Machado M, Sá S, Goldbaum T, Catania M, Campos V, Correa Giannella M, et al. In vivo response to growth hormone-releasing peptide-6 in adrenocorticotropin-dependent Cushing's syndrome by lung carcinoid tumor is associated with growth hormone secretagogue receptor type 1a mRNA expression. J Endocrinol Invest. 2007;30:334-40 pubmed
  23. Tzanela M, Karavitaki N, Stylianidou C, Tsagarakis S, Thalassinos N. Assessment of GH reserve before and after successful treatment of adult patients with Cushing's syndrome. Clin Endocrinol (Oxf). 2004;60:309-14 pubmed
    ..In the majority of these patients the disruption of GH secretion is normalized within a year after successful treatment of endogenous cortisol excess. ..
  24. Pacak K, Ilias I, Chen C, Carrasquillo J, Whatley M, Nieman L. 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;89:2214-21 pubmed
    ..As H-OCT identified a tumor in one patient with otherwise negative imaging, it should be considered only when other imaging modalities fail to localize the ACTH-secreting tumor in patients with EAS. ..
  25. Cannon J, Doherty G. A case of occult ectopic adrenocorticotropic hormone-secreting tumor: diagnostic and management dilemmas. Endocr Pract. 2008;14:588-91 pubmed
    ..Findings on biochemical evaluation were consistent with ectopic ACTH syndrome. Radiographic imaging did not clearly identify a discrete tumor...
  26. Kishida K, Moriwaki M, Miyagawa J, Okita K, Kondo T, Itoh K, et al. Successful use of 111In-pentetrotide scintigraphy for localizing ectopic adrenocorticotropin-producing bronchial carcinoid tumor in a patient with Cushing's syndrome. Intern Med. 2003;42:996-1005 pubmed
    ..This case indicates 111In-pentetrotide scintigraphy could be successfully used to identify and localize ectopic adrenocorticotropin-producing bronchial carcinoid. ..
  27. Meinardi J, van den Berg G, Wolffenbuttel B, Kema I, Dullaart R. Cyclical Cushing's syndrome due to an atypical thymic carcinoid. Neth J Med. 2006;64:23-7 pubmed
    ..Repeated measurement of urinary cortisol excretion is then required to establish or rule out the diagnosis. ..
  28. Puglia P, Caldas J, Barbosa L, Sá A, Machado M, Salgado L. [Inferior petrosal sinus catheterization: technical aspects]. Arq Bras Endocrinol Metabol. 2008;52:692-6 pubmed
    ..IPSC can be safe and successfully performed in most cases. The identification of the emissary vein of the basilar plexus and use of venography by contralateral injection, improved the method performance. ..
  29. Kim D, Park S, Choi W, Kim T, Choi Y, Jeon S, et al. Pneumocystis carinii pneumonia associated with a rapid reduction of cortisol level in a patient with ectopic ACTH syndrome treated by octreotide and ketoconazole. Exp Clin Endocrinol Diabetes. 2000;108:146-50 pubmed
    ..This case study would also like to point out that plasma ACTH and cortisol levels were decreased effectively by the combination of octreotide and ketoconazole in this instance of ectopic ACTH syndrome.
  30. Servonnet A, Delacour H, Roux X, Dehan C, Gardet V, Morand C. [Ectopic ACTH syndrome and severe hypokalaemia]. Ann Biol Clin (Paris). 2007;65:425-30 pubmed
    ..and Pseudo-Cushing's states and between the two forms of ACTH dependent Cushing's: Cushing's disease and ectopic ACTH syndrome. We report the case of a patient diagnosed with a metastatic small cells lung carcinoma associated with ..
  31. Lowry P. 60 YEARS OF POMC: Purification and biological characterisation of melanotrophins and corticotrophins. J Mol Endocrinol. 2016;56:T1-T12 pubmed publisher
    ..The review finishes with a cautionary note with regard to the misdiagnosis of the ectopic ACTH syndrome in which partial processing of ACTH can result in large concentrations of α-MSH and CLIP, which can ..
  32. Kato Y. [Beta-endorphin (beta-end)]. Nihon Rinsho. 2005;63 Suppl 8:213-5 pubmed
  33. Iwayama H, Hirase S, Nomura Y, Ito T, Morita H, Otake K, et al. Spontaneous adrenocorticotropic hormone (ACTH) normalisation due to tumour regression induced by metyrapone in a patient with ectopic ACTH syndrome: case report and literature review. BMC Endocr Disord. 2018;18:19 pubmed publisher
    ..The mechanisms of therapeutic effects of steroidogenesis inhibitors and prognosis of spontaneous remission should be elucidated further via molecular biology studies. ..
  34. Stuart S, Chandran P. Oncogenic Cushing's syndrome and nephrotic syndrome in the same patient. Am J Kidney Dis. 2001;37:E28 pubmed
    ..Oncogenic Cushing's syndrome and minimal change disease were diagnosed. We discuss the differentiation of Cushing's disease and Cushing's syndrome as well as oncogenic nephrotic syndrome and therapeutic options. ..
  35. Monaghan P, Kyriacou A, Sturgeon C, Davies A, Trainer P, White A, et al. Proopiomelanocortin interference in the measurement of adrenocorticotrophic hormone: a United Kingdom National External Quality Assessment Service study. Clin Endocrinol (Oxf). 2016;85:569-74 pubmed publisher
    ..was sufficient to detect ACTH precursors at concentrations that could be found in patients with ectopic ACTH syndrome. These data will assist laboratories in interpreting results when assessing the hypothalamic-pituitary-..
  36. Kato Y. [Lipotropin (lipotropic hormone: LPH), melanocyte-stimulating hormone (MSH)]. Nihon Rinsho. 2005;63 Suppl 8:208-10 pubmed
  37. Ujihara M, Yoshimoto T, Cho R, Totsuka Y, Imaki T, Nomura K, et al. Ectopic adrenocorticotropin syndrome exhibiting paradoxical adrenocorticotropin responsiveness to gonadotropin-releasing hormone. Endocr J. 2001;48:19-23 pubmed
    ..To our knowledge, this is the first report documenting GnRH responsiveness in ectopic ACTH syndrome.
  38. Rashid F, Riccio S, Munk P, Malfair D, Heran M. Vertebroplasty for vertebral compression fractures secondary to Cushing's syndrome induced by an ACTH-producing bronchial carcinoid tumour. Singapore Med J. 2009;50:e147-50 pubmed
    ..A bronchial carcinoid tumour was found to be the source of excess ACTH production. Three-level percutaneous vertebroplasty resulted in a marked improvement in pain. ..
  39. Kletter G, Sweetser D, Wallace S, Sawin R, Rutledge J, Geyer J. Adrenocorticotropin-secreting pancreatoblastoma. J Pediatr Endocrinol Metab. 2007;20:639-42 pubmed
    ..This malignancy is a rare cause of Cushing's syndrome, particularly at pediatric age. We describe her course including the use of ketoconazole to alleviate hypercortisolemia. ..
  40. Daousi C, Nixon T, Javadpour M, Hayden K, MacFarlane I. Inferior petrosal sinus ACTH and prolactin responses to CRH in ACTH-dependent Cushing's syndrome: a single centre experience from the United Kingdom. Pituitary. 2010;13:95-104 pubmed publisher
    ..IPSS) of ACTH with CRH stimulation helps distinguish pituitary ACTH-dependent Cushing's syndrome from the ectopic ACTH syndrome (EAS)...
  41. Vella A, Thompson G, Grant C, Van Heerden J, Farley D, Young W. Laparoscopic adrenalectomy for adrenocorticotropin-dependent Cushing's syndrome. J Clin Endocrinol Metab. 2001;86:1596-9 pubmed
    ..Twelve patients with pituitary-dependent Cushing's syndrome and four with ectopic ACTH syndrome underwent successful bilateral laparoscopic adrenalectomy...
  42. Weberpals J, Djordjevic B, Khalifa M, Oza A. A rare case of ectopic adrenocorticotropic hormone syndrome in small cell carcinoma of the vagina: a case report. J Low Genit Tract Dis. 2008;12:140-5 pubmed publisher
    ..To describe ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) to increase awareness of this condition when treating patients with gynecological small cell carcinoma (SmCC)...
  43. Newell Price J. Proopiomelanocortin gene expression and DNA methylation: implications for Cushing's syndrome and beyond. J Endocrinol. 2003;177:365-72 pubmed
    ..It is conceivable that in POMC neurons of the hypothalamus the POMC promoter is subject to a variable density of methylation with clear implications for the signalling of satiety and obesity...
  44. Dall Asta C, Santambrogio L, Castellani M, Ambrosi B. Difficulties in diagnosis and treatment of ectopic ACTH-producing tumors of the chest. Eur J Cardiothorac Surg. 2002;21:149-51 pubmed
    ..Here we present the results of different hormonal and imaging investigations and the surgical outcome of three patients with ectopic Cushing's syndrome...
  45. Findling J, Kehoe M, 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;89:6005-9 pubmed
    Inferior petrosal sinus sampling for ACTH differentiates pituitary ACTH-dependent Cushing's (CD) from the ectopic ACTH syndrome (EAS). Petrosal sinus to peripheral (IPS:P) ACTH ratios greater than 2...
  46. Uecker J, Janzow M. A case of Cushing syndrome secondary to ectopic adrenocorticotropic hormone producing carcinoid of the duodenum. Am Surg. 2005;71:445-6 pubmed
    ..This is the first reported case in the English literature of Cushing syndrome caused by ectopic ACTH production from a carcinoid of the duodenum...
  47. Khan M, Al Kahtani K, Al Ashgar H. Metastatic hepatic carcinoid associated with ectopic ACTH syndrome, resistant to octreotide and ketoconazole therapy. Ann Saudi Med. 2004;24:386-90 pubmed
  48. Ilias I, Chang R, Pacak K, Oldfield E, Wesley R, Doppman J, 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;89:3795-800 pubmed
    Bilateral sampling of the inferior petrosal sinuses (IPSS) to distinguish Cushing's disease from the ectopic ACTH syndrome is accurate but risky and technically difficult...
  49. Francia G, Davì M, Montresor E, Colato C, Ferdeghini M, Lo Cascio V. Long-term quiescence of ectopic Cushing's syndrome caused by pulmonary neuroendocrine tumor (typical carcinoid) and tumorlets: spontaneous remission or therapeutic effect of bromocriptine?. J Endocrinol Invest. 2006;29:358-62 pubmed
    ..To our knowledge, this is the second case described in literature of Cushing's syndrome caused by neuroendocrine lung tumor responsive to bromocriptine...
  50. Jiang J, Li N, Wang X, Lu Y, Bi Y, Wang W, et al. Aberrant expression and modification of silencing mediator of retinoic acid and thyroid hormone receptors involved in the pathogenesis of tumoral cortisol resistance. Endocrinology. 2010;151:3697-705 pubmed publisher
    b>Ectopic ACTH syndrome (EAS) accounts for 10-15% of cases of Cushing's syndrome and is mostly caused by small cell lung cancers or thymic carcinoids...
  51. Mengden T, Hubmann P, Muller J, Greminger P, Vetter W. Urinary free cortisol versus 17-hydroxycorticosteroids: a comparative study of their diagnostic value in Cushing's syndrome. Clin Investig. 1992;70:545-8 pubmed
    ..This simple and accurate test thus seems to be particularly useful in the outpatient evaluation of patients with suspected Cushing's syndrome...
  52. Bhansali A, Walia R, Rana S, Dutta P, Radotra B, Khandelwal N, et al. Ectopic Cushing's syndrome: experience from a tertiary care centre. Indian J Med Res. 2009;129:33-41 pubmed
    ..We describe our experience of about two decades of patients with ectopic Cushing's syndrome (ECS) seen at a tertiary care centre from north India...
  53. Benítez Velazco A, Pacheco Capote C, Latre Romero J. [Ectopic Cushing's syndrome caused by a functioning pancreatic neuroendocrine tumour in a patient with von Hippel-Lindau disease]. Rev Esp Med Nucl. 2008;27:29-33 pubmed
    ..This is the first case of Cushing's syndrome caused by an ectopic ACTH-secreting neuroendocrine tumour of the pancreas associated with von Hippel-Lindau disease...
  54. Tanaka H, Kobayashi A, Bando M, Hosono T, Tsujita A, Yamasawa H, et al. [Case of small cell lung cancer complicated with diabetes insipidus and Cushing syndrome due to ectopic adrenocorticotropic hormone secretion]. Nihon Kokyuki Gakkai Zasshi. 2007;45:793-8 pubmed
    ..Therefore, when drastic endocrinological changes are found, it is important to examine for cancer, including lung cancer, as soon as possible...
  55. Lin C, Perng W, Chen C, Lin C, Su W, Chian C. Small cell lung cancer presenting as ectopic ACTH syndrome with hypothyroidism and hypogonadism. Onkologie. 2009;32:427-30 pubmed publisher
    ..Small-cell lung cancer presenting as ectopic adrenocorticotropic hormone (ACTH) syndrome associated with hypothyroidism and hypogonadotropic hypogonadism is clinically very rare...
  56. Gasinska T, Dec R, Wichary H, Kulawik G. [Difficulties in the diagnosis of Cushing's disease--usefulness of desmopressin test]. Wiad Lek. 2007;60:80-2 pubmed
    ..This clinical case represents the demonstration of the possibility to unmask an occult eutopic Cushing's syndrome with the desmopressin test...
  57. Mizoguchi Y, Kajiume T, Miyagawa S, Okada S, Nishi Y, Kobayashi M. Steroid-dependent ACTH-produced thymic carcinoid: regulation of POMC gene expression by cortisol via methylation of its promoter region. Horm Res. 2007;67:257-62 pubmed
    ..We hypothesized that the steroid hormone may promote proopiomelanocortin (POMC) gene expression in the carcinoid cells...
  58. Tran M, Elias A. Severe myopathy and psychosis in a patient with Cushing's disease macroadenoma. Clin Neurol Neurosurg. 2003;106:1-4 pubmed
    ..Psychosis was another striking feature in this patient who during his hospital course developed multiple opportunistic infections that contributed to his demise...
  59. Oh H, Koh J, Kim M, Park J, Shong Y, Lee K, et al. A case of ACTH-producing pheochromocytoma associated with pregnancy. Endocr J. 2003;50:739-44 pubmed
    b>Ectopic ACTH syndrome is rarely caused by pheochromocytoma. We report a case of a 28-year-old woman with Cushing's syndrome due to ACTH-producing adrenal pheochromocytoma...
  60. Fuentes Tudanca S, Ortiz de Urbina P, De Dios L, Gallego Rodríguez S, Pérez Pelayo M, Del Olmo García D. [ACTH-dependent Cushing syndrome, a diagnostic challenge]. Endocrinol Nutr. 2009;56:205-8 pubmed publisher
    ..We present a case of a patient with ACTH dependent Cushing syndrome of difficult localization with usual diagnosis tests...
  61. Lalau J, Vieau D, Tenenbaum F, Westeel P, Mesmacque A, Lenne F, et al. A case of pseudo-Nelson's syndrome: cure of ACTH hypersecretion by removal of a bronchial carcinoid tumor responsible for Cushing's syndrome. J Endocrinol Invest. 1990;13:531-7 pubmed
    It may sometimes be difficult to distinguish Cushing's disease from ectopic ACTH syndrome. A case is described here of a patient with a Cushing's syndrome and diagnostic difficulties...
  62. Ogo A, Yanase T, Nawata H. [Urinary 17-ketogenic steroids (17-KGS) and 17-KGS fractions]. Nihon Rinsho. 2005;63 Suppl 8:368-72 pubmed
  63. Britvin T, Kalinin A. [ACTH-ectopic syndrome]. Klin Med (Mosk). 2003;81:8-13 pubmed
  64. Chapuis Y, Pitre J, Conti F, Abboud B, Pras Jude N, Luton J. Role and operative risk of bilateral adrenalectomy in hypercortisolism. World J Surg. 1996;20:775-9; discussion 779-80 pubmed
    ..The causes of hypercortisolism were Cushing's disease (n = 78), ectopic ACTH syndrome (n = 3), and primary adrenocortical nodular dysplasia (Carney-Meador syndrome) (n = 1)...
  65. Perrin G, Stevenaert A, Jouanneau E. [Technical aspects and surgical strategy for removal of corticotroph pituitary adenoma]. Neurochirurgie. 2002;48:186-214 pubmed
  66. Haukaas S, Halvorsen O, Nygaard S, Paus E. Cushing's syndrome in prostate cancer. An aggressive course of prostatic malignancy. Urol Int. 1999;63:126-9 pubmed
    ..The concentration of the neuroendocrine marker chromogranin A was initially within the normal range, but increased during estrogen treatment, whilst neuron-specific enolase was moderately elevated throughout the observation period...
  67. Shipley J, Schteingart D, Tandon R, Starkman M. Sleep architecture and sleep apnea in patients with Cushing's disease. Sleep. 1992;15:514-8 pubmed
  68. Donadille B, Groussin L, Waintrop C, Abbas H, Tenenbaum F, Dugue M, et al. Management of Cushing's syndrome due to ectopic adrenocorticotropin secretion with 1,ortho-1, para'-dichloro-diphenyl-dichloro-ethane: findings in 23 patients from a single center. J Clin Endocrinol Metab. 2010;95:537-44 pubmed publisher
    ..Effective treatment for the ectopic ACTH secretion syndrome (EAS) remains a therapeutic challenge. Immediate curative surgery of the responsible nonpituitary tumor is often not possible...
  69. Kumar N, Choudhary N, Kaul B, Ahlawat S. A middle aged man with acute onset quadriparesis: a diagnostic exercise. J Postgrad Med. 2006;52:308-11 pubmed
  70. de Perrot M, Spiliopoulos A, Fischer S, Totsch M, Keshavjee S. Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome. Ann Thorac Surg. 2002;73:675-81 pubmed
    ..In the future, the advent of radiologic and nuclear imaging as well as more accurate workup should help to diagnose these tumors at an earlier stage and improve the long-term outcome...
  71. Ye L, Li X, Kong X, Wang W, Bi Y, Hu L, et al. Hypomethylation in the promoter region of POMC gene correlates with ectopic overexpression in thymic carcinoids. J Endocrinol. 2005;185:337-43 pubmed
    The ectopic ACTH syndrome is caused by abnormal expression of the POMC gene product arising from non-pituitary tumors in response to the ectopic activation of the pituitary-specific promoter of this gene...
  72. Cho S, Ra Y, Lee C, Chung J, Sung S, Jheon S. Difficulties in diagnosis and management of ectopic Cushing syndrome. J Thorac Oncol. 2008;3:444-6 pubmed publisher
    ..Subsequently, a biochemical and clinical remission including hyperpigmentation was achieved...
  73. Sandeep T, Walker B. Pathophysiology of modulation of local glucocorticoid levels by 11beta-hydroxysteroid dehydrogenases. Trends Endocrinol Metab. 2001;12:446-53 pubmed
    ..These data provide encouragement that novel therapies will arise from a fuller understanding of the 11beta HSD system...
  74. Karuparthi P, Yerram P, Gupta D, Hayden M. High-cortisol states can masquerade as the cardiometabolic syndrome. J Cardiometab Syndr. 2007;2:223-6 pubmed
  75. Berardi R, Grilli G, Romagnoli E, Saladino T, Freddari F, Tamburrano T, et al. [Paraneoplastic syndromes: a review]. Clin Ter. 2005;156:281-8 pubmed
    ..This review discusses all the main paraneoplastic syndromes, focusing mainly on their clinical aspect and reminding the most commonly associated cancers...
  76. Yetkin D, Kafadar A, Gazioglu N, Oz B, Kaner G, Oguz O, et al. Kaposi sarcoma related to an ectopic hypothalamic adrenocorticotropic hormone-secreting adenoma: case report. Neurosurgery. 2009;65:E110-1; discussion E111 pubmed publisher
    ..We aim to report a case of Kaposi sarcoma (KS) with Cushing's syndrome caused by endogenic glucocorticoid-induced immunosuppression...
  77. Suzuki H. [Ectopic ACTH syndrome]. Nihon Rinsho. 2004;62:951-5 pubmed
    ..petrosal sinus sampling for ACTH with CRH administration might be a powerful tool for a diagnosis for ectopic ACTH syndrome. A variety of benign and malignant tumors of non-pituitary tissues have been associated with the ectopic ..
  78. Dahm M, Steegmüller K, Beyer J, Küstner E, Schinzel H, Lang J, et al. [Cushing syndrome in ACTH-producing neuroendocrine tumor of the hepatic duct]. Chirurg. 1993;64:662-5 pubmed
    ..Diagnostic strategy and surgical therapy in patients with neuroendocrine tumors are discussed. The diagnostic problems caused by combination with an endocrine function disorder like Cushing's syndrome are pointed out...
  79. Lieberum B, Jaspers C, Münzenmaier R. [ACTH-producing paraganglioma of the paranasal sinuses]. HNO. 2003;51:328-31 pubmed
    ..Resection of the tumour normalised ACTH and cortisol secretion...
  80. Tsagarakis S, Tsigos C, Vasiliou V, Tsiotra P, Kaskarelis J, Sotiropoulou C, et al. 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. 2002;87:1646-53 pubmed
    ..Moreover, following the combined CRH-desmopressin test only the ACTH but not the F responses were diagnostically useful, but still far from completely discriminating patients with CD and EAS...
  81. Newell Price J, Morris D, Drake W, Korbonits M, Monson J, Besser G, et al. Optimal response criteria for the human CRH test in the differential diagnosis of ACTH-dependent Cushing's syndrome. J Clin Endocrinol Metab. 2002;87:1640-5 pubmed
    ..large series reporting the response criteria that best discriminate between Cushing's disease (CD) and the ectopic ACTH syndrome (EC) when using hCRH, rather than ovine-sequence CRH...
  82. Cassier P, Abou Amara Olivieri S, Artru P, Lapalus M, Riou J, Lombard Bohas C. Mifepristone for ectopic ACTH secretion in metastatic endocrine carcinomas: report of two cases. Eur J Endocrinol. 2008;158:935-8 pubmed publisher
    ..Mifepristone clearly improved skin lesions and diabetes associated with hypercorticism. The beneficial effect lasted for about 10 months. In both cases, recurrent hypertension and hypokalemia eventually required adrenalectomy...
  83. Terzolo M, Reimondo G, Ali A, Borretta G, Cesario F, Pia A, et al. The limited value of the desmopressin test in the diagnostic approach to Cushing's syndrome. Clin Endocrinol (Oxf). 2001;54:609-16 pubmed
    ..The aim of the present study was to make a comparative evaluation of desmopressin and CRH tests in a consecutive cohort of patients with ACTH-dependent Cushing's syndrome and in a group of healthy subjects...
  84. Machado M, de Sa S, Domenice S, Fragoso M, Puglia P, Pereira M, et al. The role of desmopressin in bilateral and simultaneous inferior petrosal sinus sampling for differential diagnosis of ACTH-dependent Cushing's syndrome. Clin Endocrinol (Oxf). 2007;66:136-42 pubmed
    ..Reports on the use of desmopressin in this approach are limited. The aim of this study was to evaluate the use of desmopressin during BIPSS in a cohort of patients with ACTH-dependent Cushing's syndrome...
  85. Hernández I, Espinosa de los Monteros A, Mendoza V, Cheng S, Molina M, Sosa E, et al. Ectopic ACTH-secreting syndrome: a single center experience report with a high prevalence of occult tumor. Arch Med Res. 2006;37:976-80 pubmed
    ..In the present study we report our experience with EAS in eight patients evaluated and treated during the past 10 years...
  86. Rod A, Voicu M, Chiche L, Bazille C, Mittre H, Louiset E, et al. Cushing's syndrome associated with a nested stromal epithelial tumor of the liver: hormonal, immunohistochemical, and molecular studies. Eur J Endocrinol. 2009;161:805-10 pubmed publisher
    b>Ectopic ACTH syndrome (EAS) is principally associated with aggressive malignant tumors but also with neuroendocrine tumors of good prognosis...
  87. Shibusawa N, Mori M. [Etiology of ectopic hormone producing tumors]. Nihon Rinsho. 2004;62:865-70 pubmed
    ..We summarize etiology of 'classic' ectopic hormone producing tumors to help establish the diagnosis of paraneoplastic humoral syndromes...
  88. Moreno Fernández J, Gutiérrez Alcántara C, Gálvez Moreno M, Jimenez Reina L, Castano J, Benito Lopez P. Corticotrophin-dependent Cushing syndrome due to Sacrococcygeal Teratoma detected by [18F]fluorodeoxyglucose positron emission tomography. J Clin Endocrinol Metab. 2008;93:3282-3 pubmed publisher
  89. Nozawa A, Saitoh T, Satoh S, Nakatani Y. [A case of MEN IIA variant associated with ectopic ACTH production and mammary carcinoma]. Nihon Rinsho. 2004;62:967-71 pubmed
    ..Ectopic ACTH production was identified immunohistochemically in the pheochromecytoma. This case is a rare variant of non-familial MEN (multiple endocrine neoplasm) type IIA with ectopic ACTH production and metachronous mammary carcinoma...