Horm Metab Res 2013; 45(02): 109-117
DOI: 10.1055/s-0032-1330009
Review
© Georg Thieme Verlag KG Stuttgart · New York

The Therapy of Cushing’s Disease in Adults and Children: An Update

A. Juszczak
1   Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, UK
,
M. E. Ertorer
2   Faculty of Medicine, Adana Teaching and Research Centre – Endocrinology and Metabolism, Baskent University, Adana, Turkey
,
A. Grossman
1   Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, UK
› Institutsangaben
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Publikationsverlauf

received 17. Juli 2012

accepted 22. Oktober 2012

Publikationsdatum:
07. Dezember 2012 (online)

Abstract

Cushing’s disease (CD) is a rare endocrine disorder resulting from excessive production of adrenocorticotrophin hormone by a pituitary adenoma. The consequent hypercortisolaemia gives rise to characteristic features of the disease and its morbidities. Treatments aim to restore normal cortisol levels, provide long-term control of the disease and the tumour, and the improvement of patient well-being. The first line of treatment remains transsphenoidal surgery with remission rates of 65–90% in CD secondary to a pituitary microadenoma. Second-line treatment includes repeat surgery, radiotherapy, medical therapy, and bilateral adrenalectomy. The success rate of radiotherapy ranges from 46% to 74% and is probably independent of the mode of delivery of the radiation, but may take several years to become effective. Medical therapy is useful in acutely unwell patients or while awaiting radiotherapy to become effective. The most often-used medical agents include metyrapone and ketoconazole, which inhibit steroidogenesis; less often, centrally-acting drugs or a glucocorticoid receptor blocker are used, but experience with them is more limited. Bilateral adrenalectomy remains an important treatment option to control unresponsive severe hypercortisolism, particularly in patients with severe CD.

The management of childhood CD does not differ from adult disease, with transsphenoidal surgery as successful as in adults but radiotherapy is more rapid in onset. Regardless of the age of the patient, Cushing’s disease remains a challenge to the physician and requires a multidisciplinary approach to achieve the most desirable outcome.

 
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