Open AccessClinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization
Author(s) -
О. О. Голоунина,
Zhanna Belaya,
L Ya Rozhinskaya,
Е И Марова,
M. Yu. Pikunov,
Patimat Khandaeva,
Svetlana Arapova,
Larisa Dzeranova,
Н С Кузнецов,
В. В. Фадеев,
Г. А. Мельниченко,
Dedov
Publication year - 2021
Publication title -
terapevtičeskij arhiv
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.181
H-Index - 14
eISSN - 2309-5342
pISSN - 0040-3660
DOI - 10.26442/00403660.2021.10.201102
Subject(s) - medicine , adrenocorticotropic hormone , occult , stage (stratigraphy) , surgery , adrenalectomy , gastroenterology , hormone , pathology , paleontology , alternative medicine , biology
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.