ometendoОжирение и метаболизмObesity and metabolism2071-87132306-5524Endocrinology Research Centre10.14341/omet12876ometendo-12876Research ArticleКЛИНИЧЕСКИЙ СЛУЧАЙCASE REPORTЛечение АКТГ-эктопированного синдрома октреотидом длительного действия: эффективный контроль активности заболеванияTreatment of ACTH-ectopic syndrome with long-acting octreotide: effective control of disease activityhttps://orcid.org/0000-0002-8694-2474ГусейноваР. М.GuseinovaR. M.

Гусейнова Раисат Магомедкамиловна, ординатор

117036, Москва, ул. Дм. Ульянова, д. 11

Raisat M. Guseinova, resident

11 Dm. Ulyanova street, 117036 Moscow

rasgus-9@mail.ruhttps://orcid.org/0000-0001-6539-466XПигароваЕ. А.PigarovaE. A.

Пигарова Екатерина Александровна, д.м.н.

eLibrary SPIN: 6912-6331Scopus Author ID: 55655098500Researcher ID: T-9424-2018

Москва

Ekaterina A. Pigarova, MD, PhD

eLibrary SPIN: 6912-6331Scopus Author ID: 55655098500Researcher ID: T-9424-2018

Moscow

kpigarova@gmail.comhttps://orcid.org/0000-0002-0327-4619ДзерановаЛ. К.DzeranovaL. K.

Дзеранова Лариса Константиновна, д.м.н.

eLibrary SPIN: 2958-5555

Москва

Larisa K. Dzeranova, MD, PhD

eLibrary SPIN: 2958-5555

Moscow

dzeranovalk@yandex.ruhttps://orcid.org/0000-0003-3785-0335ШереметаМ. С.SheremetaM. S.

Шеремета Марина Сергеевна, к.м.н.

eLibrary SPIN: 7845-2194

Москва

Marina S. Sheremeta, MD, PhD

eLibrary SPIN: 7845-2194

Moscow

marina888@yandex.ruhttps://orcid.org/0000-0001-9119-2447ПржиялковскаяЕ. Г.PrzhiyalkovskayaE. G.

Пржиялковская Елена Георгиевна, к.м.н.

eLibrary SPIN: 9309-3256

Москва

Elena G. Przhiyalkovskaya, MD, PhD

eLibrary SPIN: 9309-3256

Moscow

przhiyalkovskaya.elena@gmail.comНациональный медицинский исследовательский центр эндокринологииРоссияEndocrinology Research CentreRussian Federation202208082022192180188Copyright © Гусейнова Р.М., Пигарова Е.А., Дзеранова Л.К., Шеремета М.С., Пржиялковская Е.Г., 20222022Гусейнова Р.М., Пигарова Е.А., Дзеранова Л.К., Шеремета М.С., Пржиялковская Е.Г.Guseinova R.M., Pigarova E.A., Dzeranova L.K., Sheremeta M.S., Przhiyalkovskaya E.G.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.omet-endojournals.ru/jour/article/view/12876

АКТГ-эктопированный синдром (АКТГ-ЭС) — тяжелое мультисистемное заболевание, обусловленное паранеопластической секрецией собственно АКТГ и/или, намного реже, кортиколиберина (КЛ) опухолевой тканью. Частота АКТГ-ЭС составляет 12–20% случаев эндогенного гиперкортицизма, т.е. порядка 1–2 случаев на 1 млн населения, и охватывает целый ряд новообразований: от доброкачественных неоплазм до злокачественных опухолей с распространенными метастазами, при этом чаще всего в качестве причины АКТГ-ЭС встречаются опухоли легких, поджелудочной железы и тимуса, а более редкие локализации представлены нейроэндокринными образованиями кишечника, медуллярным раком щитовидной железы, фео­хромоцитомой и мезотелиомой. Оптимальным методом лечения АКТГ-ЭС является удаление АКТГ-секретирующей опухоли. Для пациентов с неидентифицируемым источником эктопической секреции гормона вариант выбора — билатеральная адреналэктомия (так называемая «терапия отчаяния») с последующей гормонозаместительной терапией глюко- и минералокортикоидами. Варианты медикаментозной терапии, как правило, являются малоэффективным/паллиативным методом лечения. В настоящей статье представлен клинический случай успешного применения октреотида продленного действия у 36-летней женщины с тяжелым течением АКТГ-ЭС для длительного контроля паранеопластической секреции АКТГ, на фоне которого удалось достичь клинического и биохимического улучшения, сравнимого с полной ремиссией заболевания.

ACTH — ectopic syndrome (ACTH-ES) is a severe multisystem disease caused by paraneoplastic secretion of ACTH itself and/or much less often corticoliberin (CL) by tumor tissue. The frequency of ACTH-ES is 12–20% of cases of endogenous hypercortisolism, i.e. about 1–2 cases per million population, and covers a range of tumors, from benign neoplasms to malignant tumors with widespread metastases, while the most common causes of ACTH-ES are tumors of the lung, pancreas and thymus, and more rare localizations are neuroendocrine tumors (NET) of the intestine, medullary thyroid cancer, pheochromocytoma and mesothelioma. The optimal treatment for ACTH-ES is to remove the ACTH-secreting tumor. For patients with an unidentified source of ectopic hormone secretion, the choice is narrowed to bilateral adrenalectomy followed by hormone replacement therapy with glucocorticoids and mineralocorticoids. Medication options are generally a low-effective/palliative treatment option. In this article, we present a clinical case of the successful use of long-acting octreotide in a 36-year-old woman with severe ACTH-ES for long-term control of paraneoplastic ACTH secretion, against which a clinical and biochemical improvement comparable to complete remission of the disease was achieved.

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