Treatment of ACTH-ectopic syndrome with long-acting octreotide: effective control of disease activity
https://doi.org/10.14341/omet12876
Abstract
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.
About the Authors
R. M. Guseinova
Endocrinology Research Centre
Russian Federation
Russian Federation
Raisat M. Guseinova, resident
11 Dm. Ulyanova street, 117036 Moscow
E. A. Pigarova
Endocrinology Research Centre
Competing Interests:
Ekaterina A. Pigarova, MD, PhD
eLibrary SPIN: 6912-6331
Scopus Author ID: 55655098500
Researcher ID: T-9424-2018
Moscow
Competing Interests:
Пигарова Е.А. — член редакционной коллегии журнала «Ожирение и метаболизм».
L. K. Dzeranova
Endocrinology Research Centre
Competing Interests:
Larisa K. Dzeranova, MD, PhD
eLibrary SPIN: 2958-5555
Moscow
Competing Interests:
Дзеранова Л.К. — заведующая редакцией журнала «Ожирение и метаболизм».
M. S. Sheremeta
Endocrinology Research Centre
Marina S. Sheremeta, MD, PhD
eLibrary SPIN: 7845-2194
Moscow
E. G. Przhiyalkovskaya
Endocrinology Research Centre
Elena G. Przhiyalkovskaya, MD, PhD
eLibrary SPIN: 9309-3256
Moscow
References
1. Alexandraki K, Grossman A. The ectopic ACTH syndrome. Reviews in Endocrine and Metabolic Disorders. 2010;11(2):117-126. doi: https://doi.org/10.1007/s11154-010-9139-z
2. Diagnostika i lechenie neiroendokrinnykh zabolevanii. Ed. by II Dedov. Moscow: Adamant; 2003. (In Russ.).
3. Baylin S, Mendelsohn G. Ectopic (Inappropriate) Hormone Production by Tumors: Mechanisms Involved and the Biological and Clinical Implications*. Endocr Rev. 1980;1(1):45-77. doi: https://doi.org/10.1210/edrv-1-1-45
4. Ejaz S, Vassilopoulou-Sellin R, Busaidy N et al. Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion. Cancer. 2011;117(19):4381-4389. doi: https://doi.org/10.1002/cncr.26029
5. Hearn PR, Reynolds CL, Johansen K, Woodhouse NJY. Lung carcinoid with Cushing’s syndrome: control of serum acth and cortisol levels using sms 201–995 (sandostatin). Clin Endocrinol (Oxf ). 1988;28(2):181-185. doi: https://doi.org/10.1111/j.1365-2265.1988.tb03654.x
6. Rodrigues P, Castedo JL, Damasceno M, Carvalho D. Ectopic Cushing’s syndrome caused by a pulmonary ACTH-secreting tumor in a patient treated with octreotide. Arq Bras Endocrinol Metabol. 2012;56(7):461-464. doi: https://doi.org/10.1590/S0004-27302012000700009
7. De Rosa G, Testa A, Liberale I, et al. Successful treatment of ectopic Cushing’s syndrome with the long-acting somatostatin analog octreotide. Exp Clin Endocrinol. 1993;101(5):319-325. doi: https://doi.org/10.1055/s-0029-1211252
8. Bertagna X, Favrod-Coune C, Escourolle H, et al. Suppression of ectopic adrenocorticotropin secretion by the long-acting somatostatin analog octreotide. J Clin Endocrinol Metab. 1989;68(5):988-991. doi: https://doi.org/10.1210/jcem-68-5-988
9. Cheung NW, Boyages SC. Failure of somatostatin analogue to control Cushing’s syndrome in two cases of ACTH-producing carcinoid tumours. Clin Endocrinol (Oxf ). 1992;36(4):361-367. doi: https://doi.org/10.1111/j.1365-2265.1992.tb01461.x
10. Gill G, Yong A, Power E, Ramage J. Carcinoid-associated ectopic ACTH syndrome with variable response to octreotide. Postgrad Med J. 1999;75(880):98-101. doi: https://doi.org/10.1136/pgmj.75.880.98
11. Slashchuk K, Rumyantsev P, Degtyarev M, et al. Molecular Imaging of Neuroendocrine Tumors by Somatostatin-Receptor Scintigraphy (SPECT/CT) with 99mTcTektrotyd. Med Radiol Radiat Saf. 2020;65(2):44-49. (In Russ.). doi: https://doi.org/10.12737/1024-6177-2020-65-2-44-49
12. Aparicio T, Ducreux M, Baudin E et al. Antitumour activity of somatostatin analogues in progressive metastatic neuroendocrine tumours. Eur J Cancer. 2001;37(8):1014-1019. doi: https://doi.org/10.1016/s0959-8049(01)00073-9
13. Drange MR, Melmed S. Long-Acting Lanreotide Induces Clinical and Biochemical Remission of Acromegaly Caused by Disseminated Growth Hormone-Releasing Hormone-Secreting Carcinoid. J Clin Endocrinol Metab. 1998;83(9):3104-3109. doi: https://doi.org/10.1210/jcem.83.9.5088
14. Ferone D, van Hagen P, Pivonello R, et al. Physiological and pathophysiological role of somatostatin receptors in the human thymus. Eur J Endocrinol. 2000;83(9):S27-S34. doi: https://doi.org/10.1530/eje.0.143s027
15. 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: https://doi.org/10.18786/2072-0505- 2017-45-4-289-301
16. Rossijskie klinicheskie rekomendatsii. Endokrinologiya. Ed. by II Dedov, GA Mel’nichenko. Moscow: GEOTАR-Media; 2016. (In Russ.).
17. Alexandraki KI, Grossman AB. The ectopic ACTH syndrome. Rev Endocr Metab Disord. 2010;11(2):117-126. doi: https://doi.org/10.1007/s11154-010-9139-z
18. Sand M, Uecker S, Bechara FG, et al. Simultaneous ectopic adrenocorticotropic hormone syndrome and adrenal metastasis of a medullary thyroid carcinoma causing paraneoplastic Cushing’s syndrome. Int Semin Surg Oncol. 2007;4(1):15. doi: https://doi.org/10.1186/1477-7800-4-15
19. Barbosa SL, Rodien P, Leboulleux S, et al. Ectopic adrenocorticotropic hormone-syndrome in medullary carcinoma of the thyroid: a retrospective analysis and review of the literature. Thyroid. 2005;15(6):618-623. doi: https://doi.org/10.1089/thy.2005.15.618
20. Ejaz S, Vassilopoulou-Sellin R, Busaidy NL, et al. Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion: the University of Texas MD Anderson Cancer Center Experience. Cancer. 2011;117(19):4381-4389. doi: https://doi.org/10.1002/cccr.26029
21. Fallahi B, Manafi-Farid R, Eftekhari M, et al. Diagnostic fficiency of 68Ga-DOTATATE PET/CT as ompared to 99mTc-Octreotide SPECT/ CT andonventional orphologic odalities in euroendocrine umors. Asia Ocean J Nucl Med Biol. 2019;7(2):129-140. doi: https://doi.org/10.22038/AOJNMB.2019.39392.1263
22. Hardy R, Cooper M. Adrenal gland and bone. Arch Biochem Biophys. 2010;503(1):137-145. doi: https://doi.org/10.1016/j.abb.2010.06.007
23. Kaji H, Yamauchi M, Chihara K, Sugimoto T. Glucocorticoid Excess Affects Cortical Bone Geometry in Premenopausal, but not Postmenopausal, Women. Calcif Tissue Int. 2008;82(3):182-190. doi: https://doi.org/10.1007/s00223-008-9106-9
24. Lanna CMM, Paula FJA, Montenegro Jr. RM, et al. Parathyroid hormone secretion in chronic human endogenous hypercortisolism. Brazilian J Med Biol Res. 2002;35(2):229-236. doi: https://doi.org/10.1590/S0100-879X2002000200012
25. Mazziotti G, Angeli A, Bilezikian J, et al. Glucocorticoid-induced osteoporosis: an update. Trends in Endocrinology & Metabolism. 2006;17(4):144-149. doi: https://doi.org/10.1016/j.tem.2006.03.009
26. Dobnig H, Stepan JJ, Burr DB, et al. Teriparatide reduces bone microdamage accumulation in postmenopausal women previously treated with alendronate. J Bone Miner Res. 2009;24(12):1998-2006. doi: https://doi.org/10.1359/jbmr.090527
27. Holick MF, Siris ES, Binkley N, et al. Prevalence of Vitamin D inadequacy among postmenopausal North American women receiving osteoporosis therapy. J Clin Endocrinol Metab. 2005;90(6):3215-3124. doi: https://doi.org/10.1210/jc.2004-2364
28. Boonen S, Marin F, Mellstrom D, et al. Safety and efficacy of teriparatide in elderly women with established osteoporosis: bone anabolic therapy from a geriatric perspective. J Am Geriatr Soc. 2006;54(5):782-789. doi: https://doi.org/10.1111/j.1532-5415.2006.00695.x
29. Jilka RL. Molecular and cellular mechanisms of the anabolic effect of intermittent PTH. Bone. 2007;40(6):1434-1446. doi: https://doi.org/10.1016/j.bone.2007.03.017
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For citations:
Guseinova R.M., Pigarova E.A., Dzeranova L.K., Sheremeta M.S., Przhiyalkovskaya E.G. Treatment of ACTH-ectopic syndrome with long-acting octreotide: effective control of disease activity. Obesity and metabolism. 2022;19(2):180-188. (In Russ.) https://doi.org/10.14341/omet12876
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