Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization

Aim. To study the clinical, biochemical characteristics, treatment results and follow-up of patients with ectopic ACTH syndrome EAS (ACTH adrenocorticotropic hormone ). Materials and methods. A retrospective, observational, single-center study of 130 patients with EAS. Demographic information o...

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Autores principales:	Olga O. Golounina, Zhanna E. Belaya, Liudmila Ya. Rozhinskaya, Evgeniya I. Marova, Michail Yu. Pikunov, Patimat M. Khandaeva, Svetlana D. Arapova, Larisa K. Dzeranova, Nikolai S. Kuznetsov, Valentin V. Fadeev, Galina A. Melnichenko, Ivan I. Dedov
Formato:	article
Lenguaje:	RU
Publicado:	"Consilium Medicum" Publishing house 2021
Materias:	ectopic acth syndrome neuroendocrine tumor hypercortisolism bronchial carcinoid thymic carcinoid surgical treatment long-term results Medicine R
Acceso en línea:	https://doaj.org/article/61280973b0aa48b8b07cd1de3ce6bcca
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spelling	oai:doaj.org-article:61280973b0aa48b8b07cd1de3ce6bcca2021-12-01T12:22:36ZClinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization0040-36602309-534210.26442/00403660.2021.10.201102https://doaj.org/article/61280973b0aa48b8b07cd1de3ce6bcca2021-10-01T00:00:00Zhttps://ter-arkhiv.ru/0040-3660/article/viewFile/86885/65085https://doaj.org/toc/0040-3660https://doaj.org/toc/2309-5342Aim. To study the clinical, biochemical characteristics, treatment results and follow-up of patients with ectopic ACTH syndrome EAS (ACTH adrenocorticotropic hormone ). Materials and methods. A retrospective, observational, single-center study of 130 patients with EAS. Demographic information of patients, medical history, results of laboratory and instrumental investigations at the pre- and postoperative stages and follow-up of EAS were analyzed. Results. The mean age at the diagnosis ranged from 12 to 74 years (Me 40 years [28; 54]). The duration of the disease from the onset of symptoms to the verification of the diagnosis varied from 2 to 168 months (Me 17.5 months [7; 46]). Eighty-one (62,3%) patients had bronchopulmonary NET, 9 thymic carcinoid, 7 pancreatic NET, 5 pheochromocytoma, 1 cecum NET, 1 appendix carcinoid tumor, 1 medullary thyroid cancer and 25 (19.2%) had an occult source of ACTH. The median follow-up period of patients was 27 months [9.75; 61.0] with a maximum follow-up of 372 months. Currently, primary tumor was removed in 82 (63.1%) patients, bilateral adrenalectomy was performed in 23 (18%) patients, in 16 of them there was an occult source of ACTH-producing NET and in 7 patients in order to control hypercortisolism after non-successful surgical treatment. Regional and distant metastases were revealed in 25 (19.2%) patients. At the time of the last observation 59 (72%) patients were exhibited a full recovery, 12 (14.6%) had relapse of the disease and 26 (20%) died from multiple organ failure (n=18), pulmonary embolism (n=4), surgical complications (n=2), disseminated intravascular coagulation syndrome (n=1) or COVID-19 (n=1). Conclusion. In our cohort of patients bronchopulmonary NET are the most frequent cause of EAS (62.3%). Surgical treatment leads to remission of hypercortisolism in 72% cases; the proportion of relapse (14.6%) and fatal outcome (20%) remains frequent in EAS.Olga O. GolouninaZhanna E. BelayaLiudmila Ya. RozhinskayaEvgeniya I. MarovaMichail Yu. PikunovPatimat M. KhandaevaSvetlana D. ArapovaLarisa K. DzeranovaNikolai S. KuznetsovValentin V. FadeevGalina A. MelnichenkoIvan I. Dedov"Consilium Medicum" Publishing housearticleectopic acth syndromeneuroendocrine tumorhypercortisolismbronchial carcinoidthymic carcinoidsurgical treatmentlong-term resultsMedicineRRUТерапевтический архив, Vol 93, Iss 10, Pp 1171-1178 (2021)
institution	DOAJ
collection	DOAJ
language	RU
topic	ectopic acth syndrome neuroendocrine tumor hypercortisolism bronchial carcinoid thymic carcinoid surgical treatment long-term results Medicine R
spellingShingle	ectopic acth syndrome neuroendocrine tumor hypercortisolism bronchial carcinoid thymic carcinoid surgical treatment long-term results Medicine R Olga O. Golounina Zhanna E. Belaya Liudmila Ya. Rozhinskaya Evgeniya I. Marova Michail Yu. Pikunov Patimat M. Khandaeva Svetlana D. Arapova Larisa K. Dzeranova Nikolai S. Kuznetsov Valentin V. Fadeev Galina A. Melnichenko Ivan I. Dedov Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization
description	Aim. To study the clinical, biochemical characteristics, treatment results and follow-up of patients with ectopic ACTH syndrome EAS (ACTH adrenocorticotropic hormone ). Materials and methods. A retrospective, observational, single-center study of 130 patients with EAS. Demographic information of patients, medical history, results of laboratory and instrumental investigations at the pre- and postoperative stages and follow-up of EAS were analyzed. Results. The mean age at the diagnosis ranged from 12 to 74 years (Me 40 years [28; 54]). The duration of the disease from the onset of symptoms to the verification of the diagnosis varied from 2 to 168 months (Me 17.5 months [7; 46]). Eighty-one (62,3%) patients had bronchopulmonary NET, 9 thymic carcinoid, 7 pancreatic NET, 5 pheochromocytoma, 1 cecum NET, 1 appendix carcinoid tumor, 1 medullary thyroid cancer and 25 (19.2%) had an occult source of ACTH. The median follow-up period of patients was 27 months [9.75; 61.0] with a maximum follow-up of 372 months. Currently, primary tumor was removed in 82 (63.1%) patients, bilateral adrenalectomy was performed in 23 (18%) patients, in 16 of them there was an occult source of ACTH-producing NET and in 7 patients in order to control hypercortisolism after non-successful surgical treatment. Regional and distant metastases were revealed in 25 (19.2%) patients. At the time of the last observation 59 (72%) patients were exhibited a full recovery, 12 (14.6%) had relapse of the disease and 26 (20%) died from multiple organ failure (n=18), pulmonary embolism (n=4), surgical complications (n=2), disseminated intravascular coagulation syndrome (n=1) or COVID-19 (n=1). Conclusion. In our cohort of patients bronchopulmonary NET are the most frequent cause of EAS (62.3%). Surgical treatment leads to remission of hypercortisolism in 72% cases; the proportion of relapse (14.6%) and fatal outcome (20%) remains frequent in EAS.
format	article
author	Olga O. Golounina Zhanna E. Belaya Liudmila Ya. Rozhinskaya Evgeniya I. Marova Michail Yu. Pikunov Patimat M. Khandaeva Svetlana D. Arapova Larisa K. Dzeranova Nikolai S. Kuznetsov Valentin V. Fadeev Galina A. Melnichenko Ivan I. Dedov
author_facet	Olga O. Golounina Zhanna E. Belaya Liudmila Ya. Rozhinskaya Evgeniya I. Marova Michail Yu. Pikunov Patimat M. Khandaeva Svetlana D. Arapova Larisa K. Dzeranova Nikolai S. Kuznetsov Valentin V. Fadeev Galina A. Melnichenko Ivan I. Dedov
author_sort	Olga O. Golounina
title	Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization
title_short	Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization
title_full	Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization
title_fullStr	Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization
title_full_unstemmed	Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization
title_sort	clinical and laboratory characteristics and results of treatment of patients with acth-producing neuroendocrine tumors of various localization
publisher	"Consilium Medicum" Publishing house
publishDate	2021
url	https://doaj.org/article/61280973b0aa48b8b07cd1de3ce6bcca
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Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization

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