Еctopic ACTH syndrome: clinical picture, diagnosis, treatment
https://doi.org/10.14341/2306-3513-2012-1-24-36
Abstract
Diagnosis and treatment of ectopic ACTH-syndrome currently is one of the most challenging problems among other forms of endogenous hypercorticism. This syndrome is associated with presence of extrapituitary tumors characterized with different histogenesis and localization, which produce adrenocorticotropic hormone (ACTH), or – rarely – corticotrophin-releasing hormone. In most cases the ectopic synthesis of ACTH is performed in bronchial carcinoid tumors (36–46%), oat cell cancer (18–20%), medullary thyroid cancer (3–7%), pheochromocytoma (9–23%), other sites are infrequent (pancreas, thymus, parotid gland, ovaries, uterus, prostate, colon, stomach, esophagus, etc.). Much of these tumors are aggressive and are characterized with propensity to metastasize and relapse. Currently there are few contradictory data on the comparative evaluation of the effectiveness of methods of topical diagnosis of the source of ectopic ACTH-secretion, and therefore there is an urgent need to develop an optimal and most efficient algorithm for diagnostic procedures to determine the extent of the tumor in patients with ectopic ACTH-syndrome. Indications for surgery, timing and extent of surgical intervention, the effectiveness of the operation, the causes and frequency of relapses are still discussed.The present difficulties of diagnosis, as well as the lack of a unified approach to the treatment of this disease in the complex, often lead to the progression and development of a large number of serious complications functions of up to disability, which in turn does not lead to significant improvement of quality of life. Thus further research is necessary to study of this disease
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For citations:
Kuznetsov N.S., Latkina N.V., Dobreva E.A. Еctopic ACTH syndrome: clinical picture, diagnosis, treatment. Endocrine Surgery. 2012;6(1):24-36. (In Russ.) https://doi.org/10.14341/2306-3513-2012-1-24-36
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