Annals of the Russian academy of medical sciencesAnnals of the Russian academy of medical sciencesВестник Российской академии медицинских наук0869-60472414-3545"Paediatrician" Publishers LLC1267710.15690/vramn12677ENDOCRINOLOGY: CURRENT ISSUESАКТУАЛЬНЫЕ ВОПРОСЫ ЭНДОКРИНОЛОГИИReview ArticleEctopic ACTH Syndrome: Possibilities of Modern DiagnosticsЭктопический АКТГ-синдром: возможности современной диагностикиhttps://orcid.org/0000-0003-0042-76806507138145V-8221-20182703-0841GrinevaElena N.ГриневаЕлена Николаевна
Russian Federation

MD, PhD, Professor, Corresponding Member of the RAS

доктор медицинских наук, профессор, член-корреспондент РАН

grineva_en@almazovcentre.ruhttp://www.almazovcentre.ru/?page_id=15006https://orcid.org/0000-0003-4013-483157189589510v-5128-20183294-2857TsoyUliana A.ЦойУльяна Александровна
Russian Federation

MD, PhD

кандидат медицинских наук

utsoi@mail.ruhttps://orcid.org/0000-0002-7086-915366025415077567-6920RyzhkovaDaria V.РыжковаДарья Викторовна
Russian Federation

MD, PhD, Professor of the RAS

доктор медицинских наук, профессор РАН

ryzhkova_dv@almazovcentre.ruAlmazov National Medical Research CentreНациональный медицинский исследовательский центр имени В.А. Алмазова1501202479177860106202301122023Copyright ©; 2024, "Paediatrician" Publishers LLCCopyright ©; 2024, Издательство "Педиатръ"2024"Paediatrician" Publishers LLCИздательство "Педиатръ"https://vestnikramn.spr-journal.ru/jour/about/submissionshttps://vestnikramn.spr-journal.ru/jour/article/view/12677

The ectopic ACTH syndrome (EAS) is a constellation of clinical signs and symptoms caused by chronic excess production of cortisol by the adrenal glands due to stimulation by adrenocorticotropic hormone (ACTH) of extrapituitary origin. EAS is a rare pathology, it accounts for approximately 20% of all cases of ACTH-dependent hypercortisolism and up to 10% of all causes of endogenous autonomic cortisol production, however, it is often associated with severe hypercortisolism and is a life-threatening condition. The sources of ectopic secretion of ACTH are neuroendocrine tumors (NET), which differ in histological type, malignant potential, clinical course, and also have different, often difficult to detect localization. Early identification of ACTH -producing NET reduces the risk of death of the patient both from severe hypercortisolism and the tumor itself. The paper describes the features of the course of EAS, the possibilities of its diagnosis, and presents an algorithm for EAS diagnosis, created on the basis of the latest achievements of Russian and world medicine.

Эктопический АКТГ-синдром (ЭАС) представляет собой симптомокомплекс, вызванный хронической избыточной продукцией кортизола надпочечниками вследствие стимуляции их адренокортикотропным гормоном (АКТГ). ЭАС — редкая патология, он составляет примерно 20% всех случаев АКТГ-зависимого гиперкортицизма и до 10% — всех причин эндогенной автономной продукции кортизола, однако часто ассоциирован с тяжелым гиперкортицизмом и является угрожающим для жизни состоянием. Источниками эктопической секреции АКТГ выступают нейроэндокринные опухоли (НЭО), которые различаются гистологическим типом, степенью злокачественности, течением, а также имеют различную, часто трудно выявляемую локализацию. Своевременное обнаружение НЭО, продуцирующей АКТГ, снижает риск смерти больного как от тяжелого гиперкортицизма, так и от самой опухоли. В настоящей статье описаны особенности течения ЭАС, возможности его диагностики и представлен алгоритм обнаружения ЭАС, созданный на основе новейших достижений российской и мировой медицины.

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