Syndrome of ectopic ACTH secretion in a patient without a verified tumor
https://doi.org/10.14341/omet9402
Abstract
Рrevalence of endogenous hypercortisilism is about 5–6 cases per 1 million people. ACTH-dependent hypercortico- sis in the general structure is about 80%. Among this cohort of patients in 70–75% the cause of hypersecretion of ACTH is corticotrophinoma, about 10% are diagnosed with hyperplasia of corticotrophs, in the remaining 15–20% – neuroendocrine tumors of different locations, producing ACTH and / or corticoliberin. With ectopic ACTH syndrome the development of clinical manifestations is faster than with corticotropinoma, which determines the nature of the clinical picture. Difficulties in identifying the primary focus hypersecretion of ACTH may be due to its small size, absence or nonspecificity of the clinical picture from the primary focus, early metastasis in the case of malignant tumors. Besides, clinical manifestations of hyper- cortisolism can be considered and as independent nosological units, which creates certain difficulties at the stages of early diagnosis and timely treatment of ectopic ACTH syndrome.
About the Authors
Ekaterina V. ErshovaEndocrinology Research Centre
Russian Federation
MD, PhD
Evgeniya S. Senyushkina
Endocrinology Research Centre
Russian Federation
clinical resident
Ekaterina A. Troshina
Endocrinology Research Centre
Russian Federation
MD, PhD, Professor
References
1. Эндокринология: национальное руководство / Под ред. Дедова И.И., Мельниченко Г.А.. – 2-е изд., перераб. и доп. – М.: ГЭОТАР-Медиа, 2016. [Dedov II, Melnochenko GA, editors. Endokrinologiya: nacional'noe rukovodstvo. Moscow: GEHOTAR-Media; 2016. (In Russ.)]
2. Nieman LK, Biller BMK, Findling JW, et al. The Diagnosis of Cushing's Syndrome: An Endocrine Society Clinical Practice Guideline. J. Clin. Endocr. Metab. 2008;93(5):1526-1540. doi: 10.1210/jc.2008-0125.
3. Гуревич Л.Е., Воронкова И.А., Марова Е.И., и др. Клинико-морфологическая характеристика АКТГ-продуцирующих опухолей различной локализации с эктопическим синдромом Кушинга. // Альманах клинической медицины. – 2017. – № 4. – С. 289-301. [Gurevich LE, Voronkova IA, Marova EI, et al. Clinical and morphological characteristic of ACTH producing tumors of various localization and the ectopic Cushing’s syndrome. Almanac of Clinical Medicine. 2017;45(4):289-301. (in Russ.)] doi: 10.18786/2072-0505-2017-45-4-289-301.
4. Эндокринология. / Под ред. Дедова И.И., Мельниченко Г.А., Фадеева В.В. М.: ГЭОТАР-Медиа; 2009;. [Dedov II, Mel'nichenko GA, Fadeev VV, editors. Endokrinologiya. Moscow: GEOTAR-Media; 2009. (In Russ.)]
5. Brown WH. A case of pluriglandular syndrome. "Diabetes of bearded women". Lancet. 1928; 2: 1022-23.
6. Liddle GW, Nicholson WE, Island DP, et al. Clinical and laboratory studies of ectopic humoral syndromes. Recent Prog. Horm. Res. 1969; 25: 283-324.
7. Ветшев П.С., Мельниченко Г.А., Павлова М.Г., и др. Клиническое наблюдение АКТГ-эктопированного синдрома. // Хирургия. – 2006. – № 9. – С. 63-65. [Vetshev PS, Mel'nichenko GA, Pavlova MG, et al. Klinicheskoe nablyudenie AKTG-ehktopirovannogo sindroma. Hirurgiya. 2006;(9):63-65. (In Russ.)]
8. Протокол оказания медицинской помощи в рамках клинической апробации: Оказание_медицинской_помощи_пациентам_с_АКТГ-зависимым_эндогенным_гиперкортицизмом [доступ от 01.09.2018] https://www.rosminzdrav.ru/poleznye-resursy/protokoly-klinicheskoy-aprobatsii/realizuemye-protokoly-klinicheskoy-aprobatsii [интернет]
9. Гуревич Л.Е., Воронкова И.А., Марова Е.И. и соавт. Клинико-морфологическая характеристика АКТГ-продуцирующих опухолей различной локализации с эктопическим синдромом Кушинга // Альманах клинической медицины. – 2017. – Т. 45. – № 4. – С. 289–301. [Gurevich LE, Voronkova IA, Marova EI, et al. Clinical and morphological characteristic of ACTH producing tumors of various localization and the ectopic Cushing’s syndrome. Almanac of Clinical Medicine. 2017;45(4):289-301. (In Russ.)] doi: 10.18786/2072-0505-2017-45-4-289-301.
10. Российские клинические рекомендации. Эндокринология / Под ред. Дедова И.И., Мельниченко Г.А.. – М.: ГЭОТАР-Медиа; 2016. [Dedov II, Mel'nichenko GA , editors. Rossijskie klinicheskie rekomendatsii. Endokrinologiya. Moscow: GEOTАR-Media; 2016.]
11. Кузнецов Н.С., Марова Е.И., Латкина Н.В. и соавт. АКТГ-продуцирующая феохромоцитома. Клинический случай // Эндокринная хирургия. – 2012. – Т. 6. – №4. – C. 43-50. [Kuznetsov NS, Marova EI, Latkina NV, et al. ACTH-Secreting Pheochromocytoma. Case report. Endocrine Surgery. 2012;6(4):43. (In Russ.)] doi: 10.14341/2306-3513-2012-4-43-50.
12. Falhammar H, Calissendorff J, Höybye C. Frequency of Cushing’s syndrome due to ACTH-secreting adrenal medullary lesions: a retrospective study over 10 years from a single center. Endocrine. 2016;55(1):296-302. doi: 10.1007/s12020-016-1127-y.
13. Дзеранова Л.К., Михайлова Д.С., Рожинская Л.Я., и др. АКТГ-эктопированный синдром у пациентки с карциноидом легкого // Ожирение и метаболизм. – 2009. – Т. 6. – №3. – C. 54-58. [Dzeranova LK, Mikhailova DS, Rozhinskaya L, et al. ACTH-ectopic syndrome in patient with lung carcinoid. Obesity and metabolism. 2009; (3):54. (In Russ.)] doi: 10.14341/2071-8713-5246.
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1. Fig. 1. Appearance of the patient P. at the time of admission to the FSUE ENTs (November 2016) | |
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2. Fig. 2. Appearance of the patient P. after 4 months after bilateral adrenalectomy | |
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For citations:
Ershova E.V., Senyushkina E.S., Troshina E.A. Syndrome of ectopic ACTH secretion in a patient without a verified tumor. Obesity and metabolism. 2018;15(3):59-64. (In Russ.) https://doi.org/10.14341/omet9402
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