Ectopic Adrenocorticotropic Hormone Syndrome

Wajchenberg, B. L.; Mendonça, Berenice Bilharinho; Liberman, Bernardo; Pereira, Maria Adelaide Albergaria; Carneiro, Paulo Campos; Wakamatsu, Alda; Kirschner, Martin

doi:10.1210/edrv-15-6-752

Cited by 179 publications

(200 citation statements)

References 122 publications

(208 reference statements)

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“…The existence of a lung carcinoma with positive immunocytochemical staining for ACTH is the most important observation. Furthermore, a severe hypokalemic alkalosis is common in EAS and is found in 71-100% of cases of ectopic ACTH secretion, but only in 10% of cases of pituitary ACTH syndrome [9]. Serum ACTHlevels were markedly elevated and levels above 200 mol/l, as in our patient, are rare in pituitary Cushing's syndrome.…”

Section: Discussionsupporting

confidence: 49%

“…It is well known that several lung tumours like bronchial carcinoids and small cell lung carcinoma are associated with EAS in approximately 1-5% of cases [2,3]. In recent literature, bronchial carcinoids seem to be the predominant cause of EAS, while the incidence of SCLC associated with EAS seems to decrease [9]. A possible explanation for this might be the declining incidence of SCLC, apart from the fact that SCLC associated with Cushing's syndrome will be less reported due to the known association.…”

Section: Discussionmentioning

confidence: 99%

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A case of recurrent non-small-cell lung carcinoma and paraneoplastic Cushing's syndrome

et al. 2006

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Summary Secretion of ectopic adrenocorticotropic hormone (ACTH) with consequently Cushing's syndrome is a rare paraneoplastic phenomenon. It has been described in a variety of malignancies, like bronchial carcinoids, small-cell lung carcinoma, thymoma, pancreatic carcinoma and other. In many cases of suspected ectopic ACTH secretion, it is difficult to histologically or cytochemically confirm the diagnosis. We present a 63-year-old woman with a recurrent poorly differentiated squamous cell lung carcinoma with clinical and biochemical features consistent with ectopic Cushing's syndrome. Immunocytochemical staining confirmed the secretion of ACTH by tumour cells.

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Section: Discussionsupporting

confidence: 49%

Section: Discussionmentioning

confidence: 99%

A case of recurrent non-small-cell lung carcinoma and paraneoplastic Cushing's syndrome

et al. 2006

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“…Ectopic ACTH syndrome (EAS) represents 9-18% of all causes of Cushing's syndrome (1)(2)(3). EAS is mainly associated with either highly malignant tumors such as small cell lung carcinoma or less aggressive tumors such as pulmonary or mediastinal carcinoids, but also pancreatic and thymic carcinoids, gastrinoma, pheochromocytoma, and medullary thyroid cancer.…”

Section: Introductionmentioning

confidence: 99%

Cushing's syndrome associated with a nested stromal epithelial tumor of the liver: hormonal, immunohistochemical, and molecular studies

Rod

Voicu²,

Chiche³

et al. 2009

European Journal of Endocrinology

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Context: Ectopic ACTH syndrome (EAS) is principally associated with aggressive malignant tumors but also with neuroendocrine tumors of good prognosis. Recently, rare nonhepatocytic nested stromal and epithelial tumors (NSET) were characterized by their possible association with Cushing's syndrome of which biochemical and physiopathological features were still incompletely studied. Objective: To describe the clinical and hormonal characteristics of an EAS originating from a liver NSET and further understand the mechanism of cortisol overproduction. Design and setting: This is a clinical case report from the Endocrinology Department of Caen University Hospital, France. Patient and intervention: A 17-year-old female patient was found to have a large liver NSET with mild Cushingoid clinical features and intense biological hypercortisolism but moderate ACTH secretion. Resection of the tumor was curative with a 30-month follow-up. Results: The epithelial component of the tumor coexpressed ACTH mildly, corticotropin-releasing hormone (CRH) strongly, and 11b-hydroxysteroid dehydrogenase at a level comparable with normal human hepatocytes. Conclusions: Liver NSET is a new cause of EAS, which may evoke hypercortisolism by multiple biochemical pathways.

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“…B) Inibição da secreção de ACTH: a presença de receptores de somatostatina em tumores ectópicos produtores de ACTH e/ou CRH ocasionou vários relatos de terapêutica com emprego de octreotide nestes pacientes (49). A eficácia desta terapêutica parece ser dose dependente, ocorrendo escape quando a dosagem da medicação é reduzida.…”

Section: Associação Cetoconazol E Octreotide No Tratamento Do Hipercounclassified

Papel da terapia medicamentosa na síndrome de Cushing

Liberman

2003

Arq Bras Endocrinol Metab

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A terapêutica cirúrgica continua, até o momento, a ser considerada a principal, para a síndrome de Cushing endógena, qualquer que seja a causa. Entretanto em certas circunstâncias, como preparo pré-cirúrgico, ausência de cura após a cirurgia ou impossibilidade cirúrgica, a terapêutica medicamentosa tem papel importante. As drogas que inibem a esteroidogênese, como mitotane, metirapona, cetoconazol e aminoglutetimida são as drogas de escolha qualquer que seja a causa da síndrome de Cushing. Cetoconazol constitui-se em nosso meio na droga de escolha entre os inibidores da síntese de cortisol. É uma medicação que pode ser usada a longo prazo e freqüentemente não ocasiona efeitos colaterais importantes. Os chamados neuromoduladores, como ciproheptadina, bromocriptina e ácido valpróico em nossa experiência tem pouco efeito na doença de Cushing. Somatostatina de ação prolongada como Sandostatin e Sandostatin-LAR podem ter papel importante na terapêutica da síndrome de secreção ectópica de ACTH/CRH. Quimioterapia e radioterapia têm indicações especificas nas diversas causas da síndrome de Cushing.

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Ectopic Adrenocorticotropic Hormone Syndrome

Cited by 179 publications

References 122 publications

A case of recurrent non-small-cell lung carcinoma and paraneoplastic Cushing's syndrome

A case of recurrent non-small-cell lung carcinoma and paraneoplastic Cushing's syndrome

Cushing's syndrome associated with a nested stromal epithelial tumor of the liver: hormonal, immunohistochemical, and molecular studies

Papel da terapia medicamentosa na síndrome de Cushing

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