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

Abstract

Objective: Our aim was to review short-and long-term outcomes of patients treated with bilateral adrenalectomy (BADx) in ACTH-dependent Cushing's syndrome. Methods: We reviewed the literature and analysed our experience with 53 patients treated with BADx since 1990 in our institution. Results: BADx 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. Conclusion: BADx is a relatively safe and highly effective treatment, and it provides adequate control of long-term co-morbidities associated with hypercortisolism.European Journal of Endocrinology (2015) 173, M23-M32 Invited Author's profile Prof. Martin Heinrich Reincke is Director of the Medizinische Klinik and Poliklinik IV, Ludwig-MaximiliansUniversität München, and Chair of the Department of Endocrinology and Diabetology. His research specialities include adrenal physiology and pathophysiology, endocrine hypertension, pituitary disease, mineralocorticoid and glucocorticoid action and stress research. Professor Reincke heads a research team that specifically explores the prevalence and relevance of Cushing's syndrome at the epidemiological, clinical, genetic and molecular levels. The research teams of his clinic have particular expertise in assay development and validation for endocrine disease and in the development of genetically manipulated animals as models for human adrenal disease.

Prognostic factors in ectopic Cushing’s syndrome due to neuroendocrine tumors: a multicenter study

“…Furthermore, patients who underwent adrenalectomy had a better survival rate at least in the first 2 years, highlighting the importance of prompt control of hypercortisolism. Nowadays, laparoscopic adrenalectomy is a safe and minimally invasive surgical treatment (25); thus, it should be considered more frequently as an option in the management of ECS, in particular in early stages, when the hormonal syndrome is not controlled by medical treatment and/or persists after surgical removal of the primary tumor.…”

Prevalence and clinical features of the ectopic ACTH syndrome in patients with gastroenteropancreatic and thoracic neuroendocrine tumors

“…Optimal primary treatment of EAS with surgical resection of the primary tumor and its metastasis was possible in 24% of our EAS patients. In second-line treatment to control cortisol excess, cortisol included bilateral adrenalectomy, medical therapy, and radiotherapy (1,2,6,7,23,24). Ultimately, most of our patients underwent bilateral adrenalectomy to control hypercortisolism.…”

Bilateral adrenalectomy: a review of 10 years’ experience

“…[9][10][11] In the past, some investigators questioned whether partial adrenalectomy should be performed for patients with bilateral disease. This was because of concerns related to the management of patients rendered 'adrenal free' and the fear of severe hypoadrenalism encouraged some to propose partial bilateral adrenalectomy.…”