Adrenocorticotropin-Independent Bilateral Macronodular Adrenal Hyperplasia: An Unusual Cause of Cushing’s Syndrome*

Malchoff, Carl D.; Rosa, J A; Debold, C. Rowan; Kozol, Robert A.; Ramsby, Gale R.; Page, David L.; Malchoff, Diana M.; Orth, David N.

doi:10.1210/jcem-68-4-855

Cited by 89 publications

(50 citation statements)

References 21 publications

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“…The ACTH-independent types of Cushing's syndrome are caused by autonomous cortisol secretion from adrenal lesions including adenoma, carcinoma, primary micronodular dysplasia, and ACTH-independent macronodular hyperplasia [1][2][3]. Diagnosis of ACTH-independent Cushing's syndrome has become easier with advances in adrenal computed tomographic (CT) scanning and ACTH assay that demonstrates suppressed plasma ACTH levels in this syndrome.…”

mentioning

confidence: 99%

Desmopressin Stimulation Test for Diagnosis of ACTH-Dependent Cushing's Syndrome.

Shibata

Horiba

Tozawa³

et al. 1997

Endocr J

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Abstract. We evaluated the usefulness of a desmopressin (DDAVP) test in the diagnosis of ACTHdependent Cushing's syndrome. After an intravenous injection of 5 ,ug DDAVP, plasma ACTH levels increased to more than 200% of the basal levels in 10 of 10 patients with Cushing's disease, but remained less than 150% in all of 11 normal subjects, 3 patients with Addison's disease, 5 cases of Cushing's disease in remission, and 3 patients with ectopic ACTH syndrome. Peak levels of plasma cortisol after the DDAVP stimulation were 159 ± 14% in the patients with Cushing's disease, and less than 150% of the basal levels in the other 5 groups. We also found a case of Cushing's disease with periodicity which responded to DDAVP only in the active stage. In vitro studies revealed that DDAVP directly stimulates ACTH release from corticotropic adenoma cells through V1b but not V2 vasopressin receptors.In conclusion, the DDAVP stimulation test, i.e., determination of plasma ACTH levels after 5 µg DDAVP injection, seems useful for discriminating Cushing's disease from normality, and may serve to facilitate the differentiation between Cushing's disease and ectopic ACTH syndrome.

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mentioning

confidence: 99%

Desmopressin Stimulation Test for Diagnosis of ACTH-Dependent Cushing's Syndrome.

Shibata

Horiba

Tozawa³

et al. 1997

Endocr J

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“…The ACTH-independent macronodular adrenocortical hyperplasias (AIMAH) constitute a rare condition that consists of multiple bilateral adrenocortical macronodules causing a striking enlargement of the adrenal glands (Doppman et al, 1991;Malchoff et al, 1989;Swain et al, 1998). The great majority of AIMAH cases is sporadic.…”

Section: Acth-independent Macronodular Hyperplasiasmentioning

confidence: 99%

“…At the computed tomography (CT) the adrenal glands in patients with AIMAH are greatly enlarged with multiple macronodules up to 5 cm in diameter. These adrenals' weight may vary between 24 to 500g (Doppman et al, 2000;Malchoff et al, 1989).…”

Section: Aimah Diagnosis and Clinical Presentationmentioning

confidence: 99%

Adrenal Cortex Tumors and Hyperplasias

Pignatelli¹

2011

Contemporary Aspects of Endocrinology

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“…Some cases with secretion of cortisol and mineralocorticoids, cortisol and estrone and androgens only have also been reported (12)(13)(14)(15). In some patients, the adrenal lesions are found incidentally in the process of radiological investigation of another disease.…”

Section: Clinical and Laboratory Featuresmentioning

confidence: 99%

“…The bilateral adrenalectomy by overt or laparoscopic approach has been the most useful treatment in patients with AIMAH and hormonal hypersecretion (3,4,6,7,12). However, in patients with moderately increased hormonal production, unilateral adrenalectomy has been proposed as a safe and effective alternative; it is expected that, as the cell mass increases in the contralateral adrenal, a second adrenalectomy may be further necessary (85,86).…”

Section: Management Of Aimahmentioning

confidence: 99%

Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia

Costa

Lacroix

2007

Arq Bras Endocrinol Metab

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ACTH-Independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of endogenous Cushing's syndrome (CS), in which clinical features usually become apparent only after several decades of life. This form of adrenal hyperplasia typically produces excess cortisol with overt or subclinical CS, but concurrent secretion of mineralocorticoids or sexual steroids can also occur. The diagnosis is suspected by bilateral adrenal nodules larger than 1 cm on incidental imaging studies or following the demonstration of ACTH-independent hormonal hypersecretion. The pathophysiology of this entity is heterogeneous and has been intensely explored in recent years. Several G-protein coupled receptors aberrantly expressed in the adrenal cortex have been implicated in the regulation of steroidogenesis and in the initial cell proliferation in AIMAH. Several familial cases of AIMAH have been recently described with the same pattern of aberrant hormone receptors in all affected members of the family. It is probable that additional somatic genetic events related to cell cycle regulation, adhesion and transcription factors occur in addition over time in the various nodules; other mechanisms, as Gsp or ACTH receptor mutations and paracrine adrenal hormonal secretion have been rarely identified as the molecular mechanism in some cases. When systematically screened, most patients with AIMAH exhibit an in vivo aberrant cortisol response to one or various ligands suggesting the presence of aberrant adrenal receptors. The identification of these receptors creates the possibility of a specific pharmacological treatment isolated or associated with adrenalectomy. A hiperplasia adrenal macronodular independente de ACTH (AIMAH) é uma causa rara de síndrome de Cushing (SC) endógena, na qual alguns aspectos clínicos só se tornam evidentes depois de várias décadas de vida. Esta forma de hiperplasia adrenal caracteristicamente produz excesso de cortisol resultando na síndrome de Cushing franca ou subclínica, embora a secreção concomitante de mineralocorticóide, estrógeno e andrógenos também possa ocorrer. A suspeita diagnóstica é feita pela presença de nódulos adrenais bilaterais maiores que 1 cm, como achado incidental em exames de imagem ou pela demonstração de hipersecreção hormonal independente de ACTH. A fisiopatologia desta doença é heterogênea e tem sido intensamente estudada nos últimos anos. Vários receptores acoplados à proteína G, com expressão aberrante no córtex adrenal, têm sido implicados na regulação da esteroidogênese e no início da proliferação celular que ocorre na AIMAH. Diversos casos familiais de AIMAH foram recentemente descritos, e um mesmo padrão de expressão anormal dos receptores aberrantes foi observado em todos os membros afetados das famílias investigadas. Ao longo do tempo, é provável que ocorram, nos nódulos, eventos genéticos adicionais relacionados à regulação do ciclo celular, adesão e fatores de transcrição. Outros mecanismos moleculares, como mutações nos genes da proteína Gsα e do receptor de ACTH, ou ...

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Adrenocorticotropin-Independent Bilateral Macronodular Adrenal Hyperplasia: An Unusual Cause of Cushing’s Syndrome*

Cited by 89 publications

References 21 publications

Desmopressin Stimulation Test for Diagnosis of ACTH-Dependent Cushing's Syndrome.

Desmopressin Stimulation Test for Diagnosis of ACTH-Dependent Cushing's Syndrome.

Adrenal Cortex Tumors and Hyperplasias

Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia

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