A critical reappraisal of bilateral adrenalectomy for ACTH-dependent Cushing's syndrome

• Published in: European journal of endocrinology Vol. 173; no. 4; pp. M23 - M32
• Main Authors: Reincke, Martin, Ritzel, Katrin, Oßwald, Andrea, Berr, Christina, Stalla, Günter, Hallfeldt, Klaus, Reisch, Nicole, Schopohl, Jochen, Beuschlein, Felix
• Format: Journal Article
• Language: English
• Published: England Bioscientifica Ltd 01.10.2015
• Subjects: ACTH-Secreting Pituitary Adenoma - complications; ACTH-Secreting Pituitary Adenoma - metabolism; ACTH-Secreting Pituitary Adenoma - therapy; Adenoma - complications; Adenoma - metabolism; Adenoma - therapy; Adrenalectomy; Adrenocorticotropic Hormone - metabolism; Cushing Syndrome - etiology; Cushing Syndrome - metabolism; Cushing Syndrome - surgery; Glucocorticoids - therapeutic use; Hormone Replacement Therapy - methods; Humans; Pituitary ACTH Hypersecretion - etiology; Pituitary ACTH Hypersecretion - metabolism; Pituitary ACTH Hypersecretion - surgery; Pituitary Gland - metabolism; Pituitary Gland - surgery; Radiotherapy; Special Section Review; Treatment Outcome
• ISSN: 0804-4643; 1479-683X
• DOI: 10.1530/EJE-15-0265

ObjectiveOur aim was to review short- and long-term outcomes of patients treated with bilateral adrenalectomy (BADx) in ACTH-dependent Cushing's syndrome.MethodsWe reviewed the literature and analysed our experience with 53 patients treated with BADx since 1990 in our institution.ResultsBADx is considered if ACTH-dependent Cushing's syndrome is refractory to other treatment modalities. In Cushing's disease (CD), BADx is mainly used as an ultima ratio after transsphenoidal surgery and medical therapies have failed. In these cases, the time span between the first diagnosis of CD and treatment with BADx is relatively long (median 44 months). In ectopic Cushing's syndrome, the time from diagnosis to BADx is shorter (median 2 months), and BADx is often performed as an emergency procedure because of life-threatening complications of severe hypercortisolism. In both situations, BADx is relatively safe (median surgical morbidity 15%; median surgical mortality 3%) and provides excellent control of hypercortisolism; Cushing's-associated signs and symptoms are rapidly corrected, and co-morbidities are stabilised. In CD, the quality of life following BADx is rapidly improving, and long-term mortality is low. Specific long-term complications include the development of adrenal crisis and Nelson's syndrome. In ectopic Cushing's syndrome, long-term mortality is high but is mostly dependent on the prognosis of the underlying malignant neuroendocrine tumour.ConclusionBADx is a relatively safe and highly effective treatment, and it provides adequate control of long-term co-morbidities associated with hypercortisolism.