Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization
- 作者: Golounina O.1, Belaya Z.2, Rozhinskaya L.2, Marova E.2, Pikunov M.3, Khandaeva P.2, Arapova S.2, Dzeranova L.2, Kuznetsov N.2, Fadeev V.1, Melnichenko G.2, Dedov I.2
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隶属关系:
- Sechenov First Moscow State Medical University (Sechenov University)
- Endocrinology Research Centre
- Vishnevsky National Medical Research Center of Surgery
- 期: 卷 93, 编号 10 (2021)
- 页面: 1171-1178
- 栏目: Original articles
- URL: https://journals.rcsi.science/0040-3660/article/view/86885
- DOI: https://doi.org/10.26442/00403660.2021.10.201102
- ID: 86885
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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.
作者简介
Olga Golounina
Sechenov First Moscow State Medical University (Sechenov University)
编辑信件的主要联系方式.
Email: olga.golounina@mail.ru
ORCID iD: 0000-0003-2320-1051
студентка Международной школы «Медицина будущего»
俄罗斯联邦, MoscowZhanna Belaya
Endocrinology Research Centre
Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-6674-6441
д-р мед наук, зав. отд-нием нейроэндокринологии и остеопатий
俄罗斯联邦, MoscowLiudmila Rozhinskaya
Endocrinology Research Centre
Email: olga.golounina@mail.ru
ORCID iD: 0000-0001-7041-0732
д-р мед наук, проф., гл. науч. сотр. отд-ния нейроэндокринологии и остеопатий
俄罗斯联邦, MoscowEvgeniya Marova
Endocrinology Research Centre
Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-5130-4157
д-р мед наук, проф., гл. науч. сотр. отд-ния нейроэндокринологии и остеопатий
俄罗斯联邦, MoscowMichail Pikunov
Vishnevsky National Medical Research Center of Surgery
Email: olga.golounina@mail.ru
ORCID iD: 0000-0003-0559-4461
канд. мед. наук, вед. науч. сотр. отд-ния торакальной хирургии
俄罗斯联邦, MoscowPatimat Khandaeva
Endocrinology Research Centre
Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-6993-5096
канд. мед. наук, науч. сотр. отд-ния нейроэндокринологии и остеопатий
俄罗斯联邦, MoscowSvetlana Arapova
Endocrinology Research Centre
Email: olga.golounina@mail.ru
ORCID iD: 0000-0003-0028-4659
канд. мед. наук, вед. науч. сотр. отд-ния нейроэндокринологии и остеопатий
俄罗斯联邦, MoscowLarisa Dzeranova
Endocrinology Research Centre
Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-0327-4619
д-р мед наук, гл. науч. сотр. отд-ния нейроэндокринологии и остеопатий
俄罗斯联邦, MoscowNikolai Kuznetsov
Endocrinology Research Centre
Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-9419-7013
д-р мед наук, проф., зав. отд. хирургии
俄罗斯联邦, MoscowValentin Fadeev
Sechenov First Moscow State Medical University (Sechenov University)
Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-3026-6315
чл.-кор. РАН, д-р мед. наук, проф., дир. Клиники эндокринологии, зав. каф. эндокринологии №1.
俄罗斯联邦, MoscowGalina Melnichenko
Endocrinology Research Centre
Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-5634-7877
акад. РАН, д-р мед наук, проф., зам. дир. по научной работе
俄罗斯联邦, MoscowIvan Dedov
Endocrinology Research Centre
Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-8175-7886
акад. РАН, д-р мед наук, проф., президент
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