endosergЭндокринная хирургияEndocrine Surgery2306-35132310-3965Типография «Печатных дел Мастер»10.14341/2306-3513-2012-1-24-36endoserg-4012Research ArticleСтатьиArticlesАКТГ-эктопированный синдром: клиника, диагностика, лечениеЕctopic ACTH syndrome: clinical picture, diagnosis, treatmentKuznetsovN S-LatkinaN V-DobrevaE A-20121503201261№1 (2012)2436Copyright © Kuznetsov N.S., Latkina N.V., Dobreva E.A., 20122012Kuznetsov N.S., Latkina N.V., Dobreva E.A.Kuznetsov N.S., Latkina N.V., Dobreva E.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.surg-endojournals.ru/jour/article/view/4012

АКТГ-эктопированный синдром является одним из наиболее сложных в диагностическом и лечебном плане вариантов эндогенного гиперкортицизма. Развитие данного синдрома вызывают внегипофизарные опухоли различного гистогенеза и локализации, продуцирующие адренокортикотропный гормон (АКТГ), и реже – кортикотропин-рилизинг-гормон. В подавляющем большинстве случаев источником эктопической продукции АКТГ являются карциноидные опухоли бронхов (36–46%), овсяноклеточный рак легких (18–20%), медуллярный рак щитовидной железы (3–7%), феохромоцитома (9–23%). Гораздо реже встречаются опухоли других локализаций (поджелудочная железа, вилочковая и околоушная железы, яичники, матка, предстательная железа, толстая кишка, желудок, пищевод и др.). Значительная часть этих опухолей характеризуется агрессивным течением со склонностью к метастазированию и развитию рецидивов. В настоящий момент немногочисленны и противоречивы данные о сравнительной оценке эффективности методов топической диагностики в поиске очага АКТГ-эктопической секреции, в связи с чем существует острая необходимость разработки оптимального и наиболее эффективного алгоритма диагностических мероприятий для выяснения распространенности опухоли при АКТГ-эктопированном синдроме. До сих пор обсуждаются и уточняются показания к операции, сроки проведения и объем хирургического вмешательства, эффективность операции, причины и частота рецидивов. Существующие сложности диагностики, а также отсутствие единого подхода к лечению этого заболевания в комплексе нередко приводят к прогрессированию и развитию большого числа серьезных нарушений функций органов, вплоть до инвалидизации, что в свою очередь не приводит к значимому улучшению качества жизни пациента и заставляет обратить особое внимание на изучение данной патологии.

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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The authors declare that there are no conflicts of interest present.