Cushings disease - Pituitary DOI 10.1007/s11102-006-0407-6...

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Pituitary DOI 10.1007/s11102-006-0407-6 Cushing’s disease Martina De Martin · Francesca Pecori Giraldi · Francesco Cavagnini Published online: 30 October 2006 C ° Springer Science + Business Media, LLC 2006 Abstract Cushing’s disease, i.e., pituitary ACTH-secreting adenoma causing excess glucocorticoid secretion, is a rare disease with signifcant mortality and morbidity. Timely di- agnosis and appropriate treatment can alter the course oF the disease and are thereFore mandatory. ±irst step oF the diagnostic work-up is the endogenous glucocorticoid ex- cess by measurement oF urinary Free cortisol, cortisol cir- cadian rhythmicity or suppression by low doses oF dex- amethasone. In patients with equivocal results, second line tests, such as the dexamethasone-suppressed CRH test and desmopressin stimulation, usually enable the diagnosis to be confrmed. Measurement oF plasma ACTH then allows the distinction between ACTH-dependent (e.g., pituitary or ex- trapituitary neuroendocrine tumors) and ACTH-independent causes (e.g., adrenal tumors). The last step in the diagnostic algorhythm is oFten the most Fraught with problems as the distinction between Cushing’s disease and ectopic ACTH se- cretion relies on judicious interpretation oF several diagnostic procedures. Positive responses to stimulation with CRH and inhibition by high doses oF dexamethasone, iF concurrent, enable a pituitary origin to be established whereas con²ict- ing results call For inFerior petrosal sinus sampling, the latter to be perFormed in experienced centres only. Visualisation oF the tumor at pituitary imaging is helpFul but not required For the diagnosis, as microadenomas oFten remain undectected by MRI and/or CT scan and, on the other hand, visualisation oF a non-secreting incidentaloma may be misleading. Surgi- cal removal oF the pituitary tumor is the optimal treatment choice and should be attempted in every patient. Surgical M. De Martin · ±. P. Giraldi · ±. Cavagnini ( * ) Chair oF Endocrinology, University oF Milan, Ospedale San Luca, Istituto Auxologico Italiano, Milan, Italy e-mail: [email protected] Failures as well as relapses can be treated by radiotherapy, medical therapy or, iF necessary, bilateral adrenalectomy. ±i- nally, patients cured oF Cushing’s disease require long-term monitoring given the risk oF relapse and clinical burden oF associated ailments. Keywords Cushing’s disease . ACTH-secreting pituitary adenoma . CRH stimulation . InFerior petrosal sinus sampling Demographic and clinical fndings Cushing’s disease defnes the clinical picture oF an ACTH- secreting pituitary tumor, almost exclusively a benign adenoma, leading to overproduction oF glucocorticoid steroids by the adrenal cortex and clinical Features oF gluco- corticoid excess. Excess corticosteroid secretion may also be due to production oF ACTH or its releasing Factor, CRH, by an extrapituitary neuroendocrine tumor (i.e., ectopic ACTH/CRH secretion) or to primary adrenal lesions (e.g.,
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This note was uploaded on 10/14/2010 for the course ZOO 4125 taught by Professor Flanigan during the Fall '10 term at Univeristy of Wyoming- Laramie.

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Cushings disease - Pituitary DOI 10.1007/s11102-006-0407-6...

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