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PubMed · 6288366

Cushing's syndrome: problems in management.

D C Aron·J W Findling·P A Fitzgerald·P H Forsham·C B Wilson·J B Tyrrell
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Abstract

CS comprises a group of disorders characterized by hypercortisolism. The variety of causes--pituitary-dependent CS (CD), adrenal tumor, and the ectopic ACTH syndrome--necessitates a variety of therapies--surgical, radiotherapeutic, and medical. Once a specific diagnosis is made, specific therapy can be instituted. Although some controversy persists regarding treatment, particularly that of CD, for most patients it is straightforward. However, in our experience with more than 60 patients, therapeutic dilemmas can arise in a number of circumstances, e.g. the patient with the radiologically normal sella or recurrent CD after adrenalectomy. In addition, the treatment of such conditions as the large ACTH-producing pituitary tumor, Nelson's syndrome, the malignant ectopic ACTH syndrome, and adrenal carcinoma is not entirely satisfactory. Our approach to these problems is illustrated by seven cases, and we emphasize that the proper management of CS requires both correct diagnosis and the logical application of all available therapies.

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ACTH Syndrome, EctopicAdenomaAdrenal Gland NeoplasmsAdrenalectomyAdrenocorticotropic HormoneAdultCushing SyndromeFemaleHumansMaleMiddle AgedMitotaneNelson SyndromePituitary NeoplasmsRadiographyRecurrenceSella TurcicaCase ReportsJournal ArticleResearch Support, Non-U.S. Gov'tResearch Support, U.S. Gov't, P.H.S.Review

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D C Aron, J W Findling, P A Fitzgerald, P H Forsham, C B Wilson, J B Tyrrell. 1982. Cushing's syndrome: problems in management.. https://doi.org/10.1210/edrv-3-3-229

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PubMed2007

Steroid-dependent ACTH-produced thymic carcinoid: regulation of POMC gene expression by cortisol via methylation of its promoter region.

AIMS: Metyrapone causes a decrease in the serum cortisol level without affecting ACTH production in ectopic tumors. We report a case who presented with Cushing's syndrome due to an ectopic ACTH-producing thymic carcinoid. In the present case, it was demonstrated that metyrapone administration resulted in a significant decrease in the plasma ACTH and serum cortisol levels. We hypothesized that the steroid hormone may promote proopiomelanocortin (POMC) gene expression in the carcinoid cells. METHODS: An 11-year-old boy presented with Cushing's syndrome. Prior to the detection of a thymic tumor, metyrapone was administered to ameliorate the symptoms of Cushing's syndrome. Interestingly, plasma ACTH as well as serum cortisol levels immediately decreased after metyrapone administration. The levels of cortisol and ACTH were observed to be normal after complete surgical resection of the tumor. Biological characterization of the tumor cells was by in vitro analysis. RESULTS: Thein vitro culture of the tumor cells showed an increased expression of POMC in the presence of cortisol. A CpG methylation assay showed that the demethylation of the POMC promoter was induced by a steroid hormone. CONCLUSION: These findings suggest that the ectopic ACTH-producing tumor may partly be regulated by the elevated levels of cortisol.

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PubMed2007

Etomidate use for Cushing's syndrome caused by an ectopic adrenocorticotropic hormone-producing tumor.

OBJECTIVE: To report the preparation and use of etomidate in a patient with Cushing's syndrome caused by an ectopic adrenocorticotropic hormone (ACTH)-producing tumor. CASE SUMMARY: A 73-year-old man with a 5 year history of prostate cancer was admitted for symptoms consistent with Cushing's syndrome. He was started on oral metyrapone for elevated serum cortisol, ACTH, and 24 hour urinary unbound cortisol levels. Shortly after starting metyrapone, he was transferred to the medical intensive care unit for new-onset atrial fibrillation, neutropenic fever, and respiratory failure. A nasogastric tube could not be inserted to administer metyrapone. Intravenous etomidate 4 mg/h (0.06 mg/kg/h) was initiated to decrease cortisol production and provide sedation for mechanical ventilation. Despite supportive treatment, the patient died from multiple organ dysfunction. DISCUSSION: For patients exhibiting signs and symptoms of Cushing's syndrome who have no enteral access, administering etomidate intravenously may be a viable alternative treatment route. Although several articles report the use of etomidate to control cortisol overproduction, the intravenous preparation and stability are discussed only vaguely. In our patient, etomidate was infused via a 30 mL syringe, 2 mg/mL undiluted through a central venous catheter; syringe use was limited to 24 hours. Etomidate should be infused only with continuous monitoring of hemodynamics and periodic assessment of adrenal function. CONCLUSIONS: When oral or enteral medications cannot be administered and sedation is required in critically ill patients, etomidate is an appropriate intravenous agent for hypercortisolemia. There were no obvious problems with stability when undiluted etomidate 2 mg/mL was infused through a dedicated central venous catheter lumen.

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