Endogenous hypercorticism: achievements and prospects in diagnosis and treatment

Abstract

The results of examination and treatment of 647 patients with endogenous hypercortisolism were studied: pituitary corticotropinoma was detected in 494 (76.4%) patients, corticosteroma and pre — corticosteroma of the adrenal gland — in 142 (21.9%), bilateral macro-nodular hyperplasia of the adrenal glands of primary adrenal origin-in 11 (1.7%). Differential diagnosis of clinical forms of endogenous hypercortisolism was based on the assessment of the level of adrenocorticotropic hormone and cortisol, both in peripheral blood, and with selective bilateral blood sampling from the adrenal veins and lower stony sinuses, and the study of the nature of samples with 8 mg of dexamethasone. Topical diagnostics consisted in assessing the state of the adrenal glands and pituitary gland during computed tomography and magnetic resonance imaging with the use of contrast agents, and the use of special software 3D-Volume Rendering Technique allowed optimizing tactical and technical approaches to performing surgical interventions. Of the operated patients with adrenocorticotropic hormone dependent endogenous hypercortisolism, total removal according to the control magnetic resonance imaging was achieved in 92.3% of cases, subtotal — in 7.7%. However, hormonal remission was achieved only in 82.4% of cases. All patients with corticosteroma and pre-corticosteroma of the adrenal gland underwent adrenalectomy: in 6 patients by open method, in 136 patients by endovideosurgical method (in 11 patients by laparoscopic method, in 124 patients by retroperitoneoscopic method, and in 1 patient by thoracoscopic transdiaphragmatic adrenalectomy). In all patients, the operation led to recovery. Patients with benign macronodular hyperplasia of the adrenal glands needed conservative treatment with steroidogenesis blockers. Indications for surgical treatment in the volume of unilateral adrenalectomy occurred only in 2 patients.

Keywords

endogenous hypercortisolism / corticotropinoma / corticosteroma / differential diagnosis / stony sinus catheterization / transsphenoidal pituitary resection / endovideosurgical adrenalectomy

References

[1] Maystrenko NA. Vladimir Nikolayevich Shamov. (1882–1962). Grekov’s Bulletin OF Surgery. 2012;171(6):9−12. (In Russ.).

[2] Майстренко Н.А. Владимир Николаевич Шамов (1882–1962) // Вестник хирургии им. И.И. Грекова. 2012. Т. 171, № 6. С. 9−12.

[3] Maystrenko NA, Romashchenko PN, Aliyev AK. Nauchnaya shkola professora S.P. Fedorova. Saint Petersburg: Gumanistika; 2020. 263 p. (In Russ.).

[4] Майстренко Н.А., Ромащенко П.Н., Алиев А.К. Научная школа профессора С.П. Федорова. СПб.: Гуманистика, 2020. 263 c.

[5] Kalinin AP, Maystrenko NA, Khirurgiya nadpochechnikov. Moscow: Meditsina; 2000. 214 p. (In Russ.).

[6] Калинин А.П., Майстренко Н.А. Хирургия надпочечников. М.: Медицина, 2000. 214 c.

[7] Kalinin AP, Maystrenko NA, Vetshev PS. Khirurgicheskaya endokrinologiya: rukovodstvo. Saint Petersburg: Piter; 2004. 941 р. (In Russ.).

[8] Калинин А.П., Майстренко Н.А., Ветшев П.С. Хирургическая эндокринология: руководство. СПб.: Питер, 2004. 941 с.

[9] Maystrenko NA, Vavilov AG, Dovganyuk VS. Sovremennyye aspekty khirurgii nadpochechnikov. Khirurgiya. 2000;5:21–26. (In Russ.).

[10] Майстренко Н.А., Вавилов А.Г., Довганюк В.С. Современные аспекты хирургии надпочечников // Хирургия. 2000. № 5. С. 21−26.

[11] Steffensen C, Bak AM, Rubeck KZ. Epidemiology of Cushing’s syndrome. Neuroendocrinology. 2010;92(1):1–5. DOI: 10.1159/000314297

[12] Steffensen C., Bak A.M., Rubeck K.Z. Epidemiology of Cushing’s syndrome // Neuroendocrinology. 2010. Vol. 92, No. 1. P. 1–5. DOI: 10.1159/000314297

[13] Hirsch D, Tsvetov G, Manisterski Y, et al. Incidence of Cushing’s syndrome in patients with significant hypercortisoluria. Eur J Endocrinol. 2017;176(1):41–48. DOI: 10.1530/EJE-16-0631

[14] Hirsch D., Tsvetov G., Manisterski Y., et al. Incidence of Cushing’s syndrome in patients with significant hypercortisoluria // Eur J Endocrinol. 2017. Vol. 176, No. 1. P. 41–48. DOI: 10.1530/EJE-16-0631

[15] Valassi E, Franz H, Brue T, et al. ERCUSYN Study Group. Diagnostic tests for Cushing’s syndrome differ from published guidelines: data from ERCUSYN. Eur J Endocrinol. 2017;176(5): 613–624. DOI: 10.1530/EJE-16-0967

[16] Valassi E., Franz H., Brue T., et al. ERCUSYN Study Group. Diagnostic tests for Cushing’s syndrome differ from published guidelines: data from ERCUSYN // Eur J Endocrinol. 2017. Vol. 176, No. 5. P. 613–624. DOI: 10.1530/EJE-16-0967

[17] Romashchenko PN. Obosnovaniye dostupov pri endovideokhirurgicheskikh vmeshatelstvakh na nadpochechnikakh. (kliniko-anatomicheskoye issledovaniye): [dissertation] Saint Peterburg; 2000. 24 s. (In Russ.). Available from: https://search.rsl.ru/ru/record/01000264722

[18] Ромащенко П.Н. Обоснование доступов при эндовидеохирургических вмешательствах на надпочечниках. (клинико-анатомическое исследование): дис. … канд. мед. наук. СПб., 2000. Режим доступа: https://search.rsl.ru/ru/record/01000264722 Дата обращения: 09.07.21.

[19] Novoobrazovaniya nadpochechnikov. VD. Fedorova editor. Moscow: Medpraktika; 2002. (In Russ.).

[20] Новообразования надпочечников / под ред. В.Д. Федорова М.: Медпрактика, 2002.

[21] Endovideokhirurgiya nadpochechnikov. N.A. Maystrenko editor. SPb.: ELBI; 2003. (In Russ.).

[22] Эндовидеохирургия надпочечников / под ред. Н.А. Майстренко СПб. ЭЛБИ, 2003.

[23] Lacroix A, Feelders RA, Stratakis CA, et al. Cushing’s syndrome. Lancet. 2015;386(9996):913−27. DOI: 10.1016/S0140-6736(14)61375-1

[24] Lacroix A., Feelders R.A., Stratakis C.A., et al. Cushing’s syndrome // Lancet. 2015. Vol. 386, No. 9996. P. 913−927. DOI: 10.1016/S0140-6736(14)61375-1

[25] Pivonello R, Leo MDe, Cozzolino A, et al. The treatment of Cushing’s disease. Endocr Rev. 2015;36(4):385–486. DOI: 10.1210/er.2013-1048

[26] Pivonello R., Leo M.De., Cozzolino A., et al. The treatment of Cushing’s disease // Endocr Rev. 2015. Vol. 36, No. 4. P. 385–486. DOI: 10.1210/er.2013-1048

[27] Chan KC, Lit LC, Law EL, et al. Diminished urinary free cortisol excretion in patients with moderate and severe renal impairment. Clin Chem. 2004;50(4):757−769. DOI: 10.1373/clinchem.2003.029934

[28] Chan K.C., Lit L.C., Law E.L., et al. Diminished urinary free cortisol excretion in patients with moderate and severe renal impairment // Clin Chem. 2004. Vol. 50, No. 4. P. 757−769. DOI: 10.1373/clinchem.2003.029934

[29] Alwani RA, Schmit Jongbloed LW, de Jong FH, et al. Differentiating between Cushing’s disease and pseudo-Cushing’s syndrome: comparison of four tests. Eur J Endocrinol. 2014;170(4):477−86. DOI: 10.1530/EJE-13-0702

[30] Alwani R.A., Schmit Jongbloed L.W., de Jong F.H., et al. Differentiating between Cushing’s disease and pseudo-Cushing’s syndrome: comparison of four tests // Eur J Endocrinol. 2014. Vol. 170, No. 4. P. 477−486. DOI: 10.1530/EJE-13-0702

[31] Maystrenko NA, Dovganyuk VS, Fomin NF. Gormonalno-neaktivnyye opukholi nadpochechnikov. Saint Petersburg: ELBI-SPb; 2001. (In Russ.).

[32] Майстренко Н.А., Довганюк В.С., Фомин Н.Ф. «Гормонально-неактивные» опухоли надпочечников. СПб.: ЭЛБИ-СПб, 2001.

[33] Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016;175(2):1–34. DOI: 10.1530/EJE-16-0467

[34] Fassnacht M., Arlt W., Bancos I., et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors // Eur J Endocrinol. 2016. Vol. 175, No. 2. P. 1–34. DOI: 10.1530/EJE-16-0467

[35] Gusaaova NV, Savello AV, Tsoy UA, et al. Experience in the use of combined cavernous and inferior petrosal sinus sampling in the differential diagnostic of acth-dependent cushing’s syndrome. Translational Medicine. 2015;(2–3):31–32). (In Russ.).

[36] Гуссаова Н.В., Савелло А.В., Цой У.А. и др. Опыт применения катетеризации пещеристых и нижних каменистых синусов в дифференциальной диагностике АКТГ-зависимого гиперкортицизма // Трансляционная медицина. 2015. № 2–3. С. 31–32.

[37] Romashchenko PN, Zheleznyak IS, Blyumina SG, et al. Ct-planning access for adrenalectomy. Bulletin of the Russian Military Medical Academy. 2019;1:105−109. (In Russ.).

[38] Ромащенко П.Н., Железняк И.С., Блюмина С.Г. и др. КТ-планирование доступа для адреналэктомии // Вестник Российской военно-медицинской академии. 2019. № 1. С. 105−109.

[39] Dovganyuk VS. Sovremennyye tekhnologii v diagnostike i khirurgicheskom lechenii novoobrazovaniy nadpochechnikov. [dissertation] Saint Petersburg; 2005. (In Russ.) Available from: https://www.dissercat.com/content/sovremennye-tekhnologii-v-diagnostike-i-khirurgicheskom-lechenii-novoobrazovanii-nadpochechn

[40] Довганюк В.С. Современные технологии в диагностике и хирургическом лечении новообразований надпочечников: дис. … док. мед. наук. СПб., 2005. Режим доступа: https://www.dissercat.com/content/sovremennye-tekhnologii-v-diagnostike-i-khirurgicheskom-lechenii-novoobrazovanii-nadpochechn Дата обращения: 09.07.21.

[41] Bolezn Itsenko-Kushinga: klinika, diagnostika, lecheniye: рrakticheskoye rukovodstvo dlya vrachey. Dedova II, Melnichenko GA, editors. Moscow: UP Print; 2012. (In Russ.).

[42] Болезнь Иценко-Кушинга: клиника, диагностика, лечение: практическое руководство для врачей / под ред. И.И. Дедова, Г.А. Мельниченко. М.: УП Принт, 2012.

[43] Maystrenko NA. Problem aspects of diagnostics and surgical treatment of itsenko-cushing syndrome. Grekov’s Bulletin of Surgery. 2010;154(3):49−56. (In Russ.).

[44] Майстренко Н.А. Проблемные аспекты диагностики и хирургического лечения синдрома Иценко-Кушинга // Вестник хирургии им. И.И. Грекова. 2010. Т. 154, № 3. С. 49−56.

RIGHTS & PERMISSIONS

Maistrenko N.A., Romashchenko P.N., Cherebillo V.Y., Dovganyuk V.S.