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Radiotherapy as a tool for the treatment of Cushing's disease

Journal article
Authors Frederic Castinetti
Thierry Brue
Oskar Ragnarsson
Published in European Journal of Endocrinology
Volume 180
Issue 5
Pages D9-D18
ISSN 0804-4643
Publication year 2019
Published at Institute of Medicine, Department of Internal Medicine and Clinical Nutrition
Pages D9-D18
Language en
Links dx.doi.org/10.1530/eje-19-0092
Keywords term-follow-up, gamma-knife surgery, fractionated stereotactic, radiotherapy, postoperative radiation-therapy, 2nd brain-tumor, pituitary-adenomas, glucocorticoid replacement, transsphenoidal surgery, adult patients, conservative surgery, Endocrinology & Metabolism
Subject categories Endocrinology and Diabetes

Abstract

Treatment of Cushing's disease (CD) is one of the most challenging tasks in endocrinology. The first-line treatment, transsphenoidal pituitary surgery, is associated with a high failure rate and a high prevalence of recurrence. Re-operation is associated with an even higher rate of a failure and recurrence. There are three main second-line treatments for CD - pituitary radiation therapy (RT), bilateral adrenalectomy and chronic cortisol-lowering medical treatment. All these treatments have their limitations. While bilateral adrenalectomy provides permanent cure of the hypercortisolism in all patients, the unavoidable chronic adrenal insufficiency and the risk of development of Nelson syndrome are of concern. Chronic cortisol-lowering medical treatment is not efficient in all patients and side effects are often a limiting factor. RT is efficient for approximately two-thirds of all patients with CD. However, the high prevalence of pituitary insufficiency is of concern as well as potential optic nerve damage, development of cerebrovascular disease and secondary brain tumours. Thus, when it comes to decide appropriate treatment for patients with CD, who have either failed to achieve remission with pituitary surgery, or patients with recurrence, the pros and cons of all second-line treatment options must be considered.

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