Features of preparation and surgical aspects of treatment of patients with ACTH-producing neuroendocrine lung tumors

AIM: To determine significant factors affecting the survival of patients with ectopic ACTH syndrome (EAS).MATERIALS AND METHODS: A multi-center, observational study with a retrospective analysis of patients with EAS. The end point of the study was the fatal outcome of patients from various causes. In order to identify predictors of survival or mortality, univariate and multifactorial Cox regression analyses were carried out. ROC-analysis was used to determine the prognostic threshold values of individual predictors. The survival analysis was carried out using the Kaplan-Mayer method. Statistical data processing was carried out by using IBM SPSS Statistics 23.RESULTS: The age of patients at the time of diagnosis ranged from 12 to 76 years (Me 40 years [28;54]). The age of the studied population was 55 years [38; 64] for women and 42 years [32; 54] for men. The median period of observation was 50 months [13;91], with a maximum follow-up of 382 months. 92 patients (60,9%) had bronchopulmonary NET, 17 (11,3%) — thymic carcinoid, 8 — pancreatic NET, 5 –pheochromocytoma, 1– cecum NET, 1– appendix carcinoid tumor, 1 — medullary thyroid cancer and 26 (17,2%) patients had an occult NET. The primary tumor was removed in 101 patients (66,9%). Bilateral adrenalectomy was performed in 42 (27,8%) cases. Metastases were revealed in 23,2% (n=35) of patients. Relapse of the disease was observed in 24,4%, long-term remission was preserved in 64 patients (74,4%). Death occurred in 42 patients (28%). The average age of survivors was 47,0±15,2 versus 53,5±15,6 years for the deceased (p=0,022). The average survival time from diagnosis for the deceased was 32 months, Me 16,5 months [7;54]. Multivariate analysis revealed that the following factors have a direct impact on survival: age of diagnosis ≥51 years (OR 4,493; 95% CI 2,056–9,818, p<0,001), bronchopulmonary neuroendocrine tumor (NET) (OR 0,281; 95% CI 0,119–0,665, p=0,004), the presence of distant metastases (OR 2,489; 95% CI 1,141–5,427, p=0,022), late-night salivary cortisol (LNSC) ≥122,2 nmol/L (OR 2,493; 95% CI 1,014–6,128, p=0,047).CONCLUSION: The prognosis of patients with EAS is influenced by the age of diagnosis, NET localization, distant metastases and level of LNSC. The most common cause of ectopic ACTH syndrome was bronchopulmonary NET which was associated with the best survival rate.

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Survival predictors in patients with ectopic acth syndrome

Golounina

Belaya

Rozhinskaya

et al. 2022

Probl Endokrinol (Mosk)

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CT features of pulmonary ACTH-Secreting Tumor

Buryakina

Pikunov

Tarbaeva

et al. 2023

Medicinskaâ vizualizaciâ

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The purpose of the study is to improve the accuracy of CT diagnostics of pulmonary ACTH-Secreting Tumor.Material and methods. During the period from 2010 to 2022 at the Endocrinology Research Centre and the National Medical Research Center of Surgery named after A. Vishnevsky of the Ministry of Health of Russia 23 patients with pulmonary ACTH-Secreting Tumor were examined and treated. CT-features were analyzed: localization, shape, contours, connection of the formation with the bronchus, connection of the formation with the vessel, adherence to the vessel and bronchus along the long axis, structure of the neoplasm. We also analyzed the largest size of the formation, the size of the largest regional lymph node, the density of the tumor on axial sections in the native, arterial, venous and delayed phases of the study.Results. In the study, we identified a new phenotypic CT sign – the relationship with the bronchovascular bundle. The connection with the bronchus and vessel was assessed in detail. In the majority of patients, the connection with the pulmonary vessels (in most cases with small bronchial arteries) was especially clearly noted: an intimate attachment of the tumor along its long axis to the vessel. Less often it was possible to trace the connection with the bronchus.Conclusion. ACTH-producing NET of the lung is most often a peripherally located oval-shaped formation with even/ lobulated contours, adjacent to the bronchovascular bundle along the long axis of the tumor, intensively enchancing (increase of density parameters by more than 40 HU compared with the unenhanced phase) in the venous, less often the arterial phase of bolus contrast enhancement.

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Efficacy and predictors of response to somatostatin analogues in patients with ectopic ACTH syndrome

Golounina,

Belaya,

Markovich

et al. 2024

Terapevticheskii arkhiv

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Aim. To study the immediate efficacy, tolerability and predictors of response to prolonged-acting somatostatin analogues (SSAs) in patients with ectopic ACTH syndrome (EAS). Materials and methods. A multicenter, observational study with a retrospective analysis. The effectiveness of treatment was evaluated every 12–24 weeks by the activity of hypercortisolism, clinical symptoms of the disease, control of tumor growth. Patients were conditionally divided into “responders” and “non-responders” at time points of 6, 12 and 24 months. Radiological efficacy was performed according to the RECIST 1.1. Statistical data processing was carried out by using IBM SPSS Statistics 23. Results. The study included 46 patients (26 women, 20 men). The median follow-up period was 71 months [32; 122]. Localized tumor process (T1-3N0M0) – in 19 (41.3%) patients, locally widespread (T1-3N1M0, T4N0-1M0) – in 15 (32.6%), generalized (T1-4N0-1M1) – in 12 (26.1%) cases. Surgical treatment was performed in 33 (71.7%) patients. SSAs were the first-line therapy for all 46 patients. The average duration of SSAs use was 34 months (Me 27.5 [13.8; 41]). Dose escalation was required in 39.4% cases, the period before the first dose escalation was on average 9 months. 13 (48.1%) patients achieved drug-induced remission of the disease. Normalization of cortisol in 24hUFC or its decrease ≥50% from the baseline level by the 6th month of treatment was achieved in 46.4%, by the 12th month – in 61.9%, “eluding” from the effect of therapy was observed in 3 patients. Objective response was evaluated in 30 patients. There was no complete response to the treatment. In 4 (13.3%) cases – a partial response, in 15 (50%) – disease stabilization, in 11 (36.7%) – disease progression. The best therapeutic efficacy of SSAs is associated with bronchial NET (HR 0.078, 95% CI 0.010–0.633; p=0.017), and resistance to treatment is associated with negative expression of SSTR5 (HR 6.532, 95% CI 1.019–41.878; p=0.048). Conclusion. SSAs are the drugs of choice for the first-line treatment of patients with EAS, allowing for long-term effective control of hypercortisolism and tumor progression in more than 60% of patients. Significant factors determining the response to SSAs after 6 months of treatment are expression status of SSTR5 and NET localization.

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Features of preparation and surgical aspects of treatment of patients with ACTH-producing neuroendocrine lung tumors

Cited by 3 publications

References 17 publications

Survival predictors in patients with ectopic acth syndrome

Survival predictors in patients with ectopic acth syndrome

CT features of pulmonary ACTH-Secreting Tumor

Efficacy and predictors of response to somatostatin analogues in patients with ectopic ACTH syndrome

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