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

• Published in: Terapevtic̆eskii arhiv Vol. 93; no. 10; pp. 1171 - 1178
• Main Authors: Golounina, Olga O., Belaya, Zhanna E., Rozhinskaya, Liudmila Ya, Marova, Evgeniya I., Pikunov, Michail Yu, Khandaeva, Patimat M., Arapova, Svetlana D., Dzeranova, Larisa K., Kuznetsov, Nikolai S., Fadeev, Valentin V., Melnichenko, Galina A., Dedov, Ivan I.
• Format: Journal Article
• Language: English; Russian
• Published: "Consilium Medicum" Publishing house 15.10.2021
• Subjects: bronchial carcinoid; ectopic acth syndrome; hypercortisolism; long-term results; neuroendocrine tumor; surgical treatment; thymic carcinoid
• ISSN: 0040-3660; 2309-5342
• DOI: 10.26442/00403660.2021.10.201102

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.