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

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Abstract

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.

About the authors

Olga O. Golounina

Sechenov First Moscow State Medical University (Sechenov University)

Author for correspondence.
Email: olga.golounina@mail.ru
ORCID iD: 0000-0003-2320-1051

студентка Международной школы «Медицина будущего»

Russian Federation, Moscow

Zhanna E. 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

Russian Federation, Moscow

Liudmila Ya. 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

Russian Federation, Moscow

Evgeniya I. 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

Russian Federation, Moscow

Michail Yu. 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

Russian Federation, Moscow

Patimat M. 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

Russian Federation, Moscow

Svetlana D. 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

Russian Federation, Moscow

Larisa K. 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

Russian Federation, Moscow

Nikolai S. Kuznetsov

Endocrinology Research Centre

Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-9419-7013

д-р мед наук, проф., зав. отд. хирургии

Russian Federation, Moscow

Valentin V. 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.

Russian Federation, Moscow

Galina A. 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

Russian Federation, Moscow

Ivan I. Dedov

Endocrinology Research Centre

Email: olga.golounina@mail.ru
ORCID iD: 0000-0002-8175-7886

акад. РАН, д-р мед наук, проф., президент

Russian Federation, Moscow

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Supplementary files

Supplementary Files
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2. Fig. 1. The prevalence of complications of endogenous hypercortisolism in patients with ACTH-ES in the active stage of the disease.

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3. Fig. 2. Localization of the source of ACTH-producing NETs.

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4. Fig. 3. The structure of surgical interventions in patients with ACTH-ES (n=82).

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5. Fig. 4. Status of patients with ACTH-ES at the time of the last visit.

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6. Fig. 5. The structure of the causes of death in patients with ACTH-ES (n=26).

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