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1

Llewellyn, David C., Rajaventhan Srirajaskanthan, Royce P. Vincent, et al. "Calcitonin-secreting neuroendocrine neoplasms of the lung: a systematic review and narrative synthesis." Endocrine Connections 10, no. 4 (2021): 447–61. http://dx.doi.org/10.1530/ec-21-0071.

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Calcitonin-secreting neuroendocrine neoplasms of the lung are rare, with few cases reported in the literature. Differentiating between medullary thyroid carcinoma and an ectopic source of calcitonin secretion can represent a complex diagnostic conundrum for managing physicians, with cases of unnecessary thyroidectomy reported in the literature. This manuscript reports a case of ectopic hypercalcitonaemia from a metastatic neuroendocrine neoplasm of the lung with concurrent thyroid pathology and summarises the results of a systematic review of the literature. Medical Literature Analysis and Ret
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2

Ronot, Maxime, and Marie-Pierre Vullierme. "Morphological imaging of gastrointestinal and lung neuroendocrine neoplasms." Current Opinion in Endocrine and Metabolic Research 19 (August 2021): 1–7. http://dx.doi.org/10.1016/j.coemr.2021.05.001.

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Michalak, Slawomir, Joanna Rybacka-Mossakowska, Wojciech Ambrosius, et al. "The Markers of Glutamate Metabolism in Peripheral Blood Mononuclear Cells and Neurological Complications in Lung Cancer Patients." Disease Markers 2016 (2016): 1–8. http://dx.doi.org/10.1155/2016/2895972.

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Objective. To evaluate the involvement of glutamate metabolism in peripheral blood mononuclear cells (PBMC) in the development of neurological complications in lung cancer and during chemotherapy. Methods. The prospective study included 221 lung cancer patients treated with chemotherapeutics. Neurological status and cognitive functions were evaluated at baseline and after 6-month follow-up. Glutamate level, the activities of glutaminase- (GLS-) glutamate synthetizing enzyme, glutamate dehydrogenase (GDH), and glutamate decarboxylase catalyzing glutamate degradation were analyzed in PBMC and in
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4

Couvelard, Anne, Jérôme Cros, Atsuko Kasajima, Günter Klöppel, and Aurélie Cazes. "Digestive and lung high-grade neuroendocrine neoplasms: Update and challenging issues." Current Opinion in Endocrine and Metabolic Research 18 (June 2021): 224–29. http://dx.doi.org/10.1016/j.coemr.2021.04.002.

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Wong, Cheuk Lik, Matthew Tai Fai Kwan, Sammy Pak Lam Chen, Mei Tik Leung, Tim Wai Chan, and Vicki Ho Kee Tam. "ODP049 Oncocytic adrenocortical carcinoma misdiagnosed as renal oncocytoma and use of urinary steroid profiling in disease monitoring after mitotane therapy." Journal of the Endocrine Society 6, Supplement_1 (2022): A62. http://dx.doi.org/10.1210/jendso/bvac150.128.

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Abstract Introduction Oncocytic adrenocortical carcinoma (OAC) is a rare, more indolent variant of adrenocortical carcinoma (ACC). With fewer than 70 cases reported in the English literature, the biological behavior and its response to treatment are poorly characterized. The distinction of OAC from other oncocytic neoplasms (e. g., kidneys) and variants of renal cell carcinoma (RCC) may pose diagnostic challenge pathologically. Urine steroid profiling (USP) of OAC has not been previously reported. Case description A 65-year-old Chinese man was incidentally found to have a large left upper abdo
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6

Modica, Roberta, Anna La Salvia, Alessia Liccardi, et al. "Lipid Metabolism and Homeostasis in Patients with Neuroendocrine Neoplasms: From Risk Factor to Potential Therapeutic Target." Metabolites 12, no. 11 (2022): 1057. http://dx.doi.org/10.3390/metabo12111057.

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Lipid metabolism is known to be involved in tumorigenesis and disease progression in many common cancer types, including colon, lung, breast and prostate, through modifications of lipid synthesis, storage and catabolism. Furthermore, lipid alterations may arise as a consequence of cancer treatment and may have a role in treatment resistance. Neuroendocrine neoplasms (NENs) are a heterogeneous group of malignancies with increasing incidence, whose mechanisms of cancer initiation and progression are far from being fully understood. Alterations of lipid metabolism may be common across various can
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7

Espinosa-Olarte, Paula, Anna La Salvia, Maria C. Riesco-Martinez, Beatriz Anton-Pascual, and Rocio Garcia-Carbonero. "Chemotherapy in NEN: still has a role?" Reviews in Endocrine and Metabolic Disorders 22, no. 3 (2021): 595–614. http://dx.doi.org/10.1007/s11154-021-09638-0.

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AbstractNeuroendocrine neoplasms (NENs) comprise a broad spectrum of tumors with widely variable biological and clinical behavior. Primary tumor site, extent of disease, tumor differentiation and expression of so matostatin receptors, proliferation and growth rates are the major prognostic factors that determine the therapeutic strategy. Treatment options for advanced disease have considerably expanded in recent years, particularly for well differentiated tumors (NETs). Novel drugs approved over the past decade in this context include somatostatin analogues and 177Lu-oxodotreotide for somatost
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8

Kasajima, Atsuko, and Günter Klöppel. "Neuroendocrine neoplasms of lung, pancreas and gut: a morphology-based comparison." Endocrine-Related Cancer 27, no. 11 (2020): R417—R432. http://dx.doi.org/10.1530/erc-20-0122.

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The bronchopulmonary (BP) and gastroenteropancreatic (GEP) organ systems harbor the majority of the neuroendocrine neoplasms (NENs) of the body, comprising 20 and 70% of all NENs, respectively. Common to both NEN groups is a classification distinguishing between well- and poorly differentiated NENs associated with distinct genetic profiles. Differences between the two groups concern the reciprocal prevalence of well and poorly differentiated neoplasms, the application of a Ki67-based grading, the variety of histological patterns, the diversity of hormone expression and associated syndromes, th
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9

Volante, Marco, Ozgur Mete, Giuseppe Pelosi, Anja C. Roden, Ernst Jan M. Speel, and Silvia Uccella. "Molecular Pathology of Well-Differentiated Pulmonary and Thymic Neuroendocrine Tumors: What Do Pathologists Need to Know?" Endocrine Pathology 32, no. 1 (2021): 154–68. http://dx.doi.org/10.1007/s12022-021-09668-z.

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AbstractThoracic (pulmonary and thymic) neuroendocrine tumors are well-differentiated epithelial neuroendocrine neoplasms that are classified into typical and atypical carcinoid tumors based on mitotic index cut offs and presence or absence of necrosis. This classification scheme is of great prognostic value but designed for surgical specimens, only. Deep molecular characterization of thoracic neuroendocrine tumors highlighted their difference with neuroendocrine carcinomas. Neuroendocrine tumors of the lung are characterized by a low mutational burden, and a high prevalence of mutations in ch
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10

Kalkanis, Alexandros, Dimitrios Papadopoulos, Dries Testelmans, Alexandra Kopitopoulou, Eva Boeykens, and Els Wauters. "Bronchoalveolar Lavage Fluid-Isolated Biomarkers for the Diagnostic and Prognostic Assessment of Lung Cancer." Diagnostics 12, no. 12 (2022): 2949. http://dx.doi.org/10.3390/diagnostics12122949.

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Lung cancer is considered one of the most fatal malignant neoplasms because of its late detection. Detecting molecular markers in samples from routine bronchoscopy, including many liquid-based cytology procedures, such as bronchoalveolar lavage fluid (BALF), could serve as a favorable technique to enhance the efficiency of a lung cancer diagnosis. BALF analysis is a promising approach to evaluating the tumor progression microenvironment. BALF’s cellular and non-cellular components dictate the inflammatory response in a cancer-proliferating microenvironment. Furthermore, it is an essential mate
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11

Al-Toubah, Taymeyah, Michael J. Schell, Mauro Cives, Jun-Min Zhou, Heloisa P. Soares, and Jonathan R. Strosberg. "A Phase II Study of Ibrutinib in Advanced Neuroendocrine Neoplasms." Neuroendocrinology 110, no. 5 (2019): 377–83. http://dx.doi.org/10.1159/000502383.

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Background: Ibrutinib is an orally administered inhibitor of Bruton’s tyrosine kinase (Btk). Preclinical data suggest that mast cells are recruited within neuroendocrine neoplasms (NENs) where they stimulate angiogenesis and tumor growth. Ibrutinib inhibits mast cell degranulation and has been associated with regression of tumors in a mouse insulinoma model. Methods: A prospective, phase II trial evaluated patients with advanced gastrointestinal (GI)/lung NENs and pancreatic NENs (pNENs) who had evidence of progression within 12 months of study entry on at least one prior therapy. Patients rec
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12

Capdevila, Jaume, Lisa Bodei, Philippa Davies, et al. "Unmet Medical Needs in Metastatic Lung and Digestive Neuroendocrine Neoplasms." Neuroendocrinology 108, no. 1 (2018): 18–25. http://dx.doi.org/10.1159/000493319.

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Unmet medical needs are not infrequent in oncology, and these needs are usually of higher magnitude in rare cancers. The field of neuroendocrine neoplasms (NENs) has evolved rapidly during the last decade, and, currently, a new WHO classification is being implemented and several treatment options are available in the metastatic setting after the results of prospective phase III clinical trials. However, several questions are still unanswered, and decisions in our daily clinical practice should be made with limited evidence. In the 2016 meeting of the advisory board of the European Neuroendocri
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13

Sinvani, Naama Peltz, Ruth Percik, Inbal Uri, Sapir Kon Kfir, Amir Tirosh, and Amit Tirosh. "Low Risk for All-Cause Mortality Among Patients With Lung Neuroendocrine Tumors Co-Diagnosed With Pituitary Adenoma." Journal of the Endocrine Society 5, Supplement_1 (2021): A646. http://dx.doi.org/10.1210/jendso/bvab048.1316.

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Abstract Context: Lung neoplasm often co-occur with pituitary adenoma (PA). However, whether co-diagnosis of lung neuroendocrine tumors (LNET) and PA constitute unique entity, and the impact of such co-diagnosis on patients outcome is yet to be defined. Objective: To compare the clinical characteristics of patients with LNET to those co-diagnosed with PA. Design: Retrospective, case-control study including patients diagnosed with LNET or PA between 2000 and 2016. Setting: The Surveillance, Epidemiology and End Results database. Patients: 2,947 patients had LNET, including 2,913 with LNET alone
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14

Modlin, Irvin M., Mark Kidd, Lisa Bodei, Anna Malczewska, Alexandra Kitz, and Kjell Oberg. "Circulating biomarkers of gastroenteropancreatic and lung neuroendocrine neoplasms: “The times they are a changin”." Current Opinion in Endocrine and Metabolic Research 18 (June 2021): 243–53. http://dx.doi.org/10.1016/j.coemr.2021.03.017.

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15

Ragni, Alberto, Alice Nervo, Mauro Papotti, et al. "Pituitary metastases from neuroendocrine neoplasms: case report and narrative review." Pituitary 24, no. 5 (2021): 828–37. http://dx.doi.org/10.1007/s11102-021-01178-9.

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Abstract Purpose Pituitary metastases (PM) are uncommon findings and are mainly derived from breast and lung cancers. No extensive review of PM from neuroendocrine neoplasms (NENs) is on record. Here we describe a clinical case of PM from pancreatic NEN and review the clinical features of PM from NENs reported in the literature. Methods A case of PM from a pancreatic NEN followed at our institution is described. We also reviewed the 43 cases of PM from NENs reported in the literature. Results A 59-year old female patient, previously submitted to duodeno-cephalo-pancreasectomy for a well-differ
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16

Vélayoudom-Céphise, Fritz-Line, Pierre Duvillard, Lydia Foucan, et al. "Are G3 ENETS neuroendocrine neoplasms heterogeneous?" Endocrine-Related Cancer 20, no. 5 (2013): 649–57. http://dx.doi.org/10.1530/erc-13-0027.

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The new WHO classification of gastroenteropancreatic (GEP) neuroendocrine tumors (NET) implies that G3 neoplasms with mitotic index >20 and/or Ki67 index >20% are neuroendocrine carcinomas (NEC), described as poorly differentiated, small or large cell types, by analogy with lung NEC. To characterize the subgroup of non-small-cell-type GEP and thoracic NET with mitotic index >20 and/or Ki67 >20% according to their pathological features, response to cisplatin and overall survival (OS). We reviewed pathological and clinical presentation of G3 non-small-cell-type NET referred to our in
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17

Telo, Silvi, Letizia Calderoni, Sara Vichi, Federico Zagni, Paolo Castellucci, and Stefano Fanti. "Alternative and New Radiopharmaceutical Agents for Lung Cancer." Current Radiopharmaceuticals 13, no. 3 (2020): 185–94. http://dx.doi.org/10.2174/1874471013666191223151402.

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Background: FDG PET/CT imaging has an established role in lung cancer (LC) management. Whilst it is a sensitive technique, FDG PET/CT has a limited specificity in the differentiation between LC and benign conditions and is not capable of defining LC heterogeneity since FDG uptake varies between histotypes. Objective: To get an overview of new radiopharmaceuticals for the study of cancer biology features beyond glucose metabolism in LC. Methods: A comprehensive literature review of PubMed/Medline was performed using a combination of the following keywords: “positron emission tomography”, “lung
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18

Scoazec, Jean-Yves. "Lung and digestive neuroendocrine neoplasms. From WHO classification to biomarker screening: Which perspectives?" Annales d'Endocrinologie 80, no. 3 (2019): 163–65. http://dx.doi.org/10.1016/j.ando.2019.04.011.

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19

Breitling, Lutz Philipp, Anja Rinke, and Thomas Mathias Gress. "Recent Survival Trends in High-Grade Neuroendocrine Neoplasms and Lung Cancer." Neuroendocrinology 110, no. 3-4 (2019): 225–33. http://dx.doi.org/10.1159/000500883.

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Background: Poorly differentiated neuroendocrine neoplasms (pdNEN) are a rare cancer entity, treatment of which is to a great part informed by studies on the much more common small-cell lung cancer (SCLC). Objective: To reveal and compare recent survival trends for pdNEN and SCLC in an authorative, population-based database. Methods: Using the Surveillance, Epidemiology, and End Results 18 database, 3,482 digestive tract pdNEN and 30,383 SCLC diagnosed from 2000 through 2015 were analyzed in detail. Results: Whereas changes in one- and 2-year relative survival in pdNEN were small, improvements
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20

Ramos, Andrés Felipe García, Jose Leonel Zambrano Urbano, Víctor Manuel Blanco Pico, et al. "Brain Metastases in a Patient With Papillary Thyroid Carcinoma. Clinical Case Report." Journal of the Endocrine Society 5, Supplement_1 (2021): A883—A884. http://dx.doi.org/10.1210/jendso/bvab048.1804.

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Abstract Introduction: Papillary thyroid carcinoma (PTC) is the most common neoplasm of the endocrine system and the least aggressive, representing between 70% and 80% of thyroid neoplasms. This is characterized by slow growth and a low risk of progression to metastasis. Brain Metastases (BM) associated with PTC occur with a incidence rate of 0.15%. Tyrosine kinase inhibitors (TKI) are a very promising alternative for managing these patients. Here is described the case of a patient with PTC with brain and lung metastases, her respective management and evolution. Clinical Case Report: A 64-year
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21

Kiriakopoulos, Andreas, Periklis Giannakis, and Evangelos Menenakos. "Calcitonin: current concepts and differential diagnosis." Therapeutic Advances in Endocrinology and Metabolism 13 (January 2022): 204201882210993. http://dx.doi.org/10.1177/20420188221099344.

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Calcitonin (CT) is most effectively produced by the parafollicular cells of the thyroid gland. It acts through the calcitonin receptor (CTR), a seven-transmembrane class II G-protein-coupled receptor linked to multiple signal transduction pathways with its main secretagogues being calcium and gastrin. It is clinically used mostly in the diagnosis and follow-up of medullary thyroid carcinoma (MTC). Hypercalcitoninemia can be attributed to primary (e.g. CT-secreting tumor) or secondary (e.g. due to hypercalcemia) overproduction, underexcretion (e.g. renal insufficiency), drug reaction (e.g. β-bl
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Angelousi, Anna, Eva Kassi, Narjes Ansari-Nasiri, Harpal Randeva, Gregory Kaltsas, and George Chrousos. "Clock genes and cancer development in particular in endocrine tissues." Endocrine-Related Cancer 26, no. 6 (2019): R305—R317. http://dx.doi.org/10.1530/erc-19-0094.

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Circadian rhythms at a central and peripheral level are operated by transcriptional/translational feedback loops involving a set of genes called ‘clock genes’ that have been implicated in the development of several diseases, including malignancies. Dysregulation of the Clock system can influence cancer susceptibility by regulating DNA damage and repair mechanisms, as well as apoptosis. A number of oncogenic pathways can be dysregulated via clock genes’ epigenetic alterations, including hypermethylation of clock genes’ promoters or variants of clock genes. Clock gene disruption has been studied
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23

Venizelos, Andreas, Hege Elvebakken, Aurel Perren, et al. "The molecular characteristics of high-grade gastroenteropancreatic neuroendocrine neoplasms." Endocrine-Related Cancer 29, no. 1 (2022): 1–14. http://dx.doi.org/10.1530/erc-21-0152.

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High-grade (HG) gastroenteropancreatic (GEP) neuroendocrine neoplasms (NEN) are rare but have a very poor prognosis and represent a severely understudied class of tumours. Molecular data for HG GEP-NEN are limited, and treatment strategies for the carcinoma subgroup (HG GEP-NEC) are extrapolated from small-cell lung cancer (SCLC). After pathological re-evaluation, we analysed DNA from tumours and matched blood samples from 181 HG GEP-NEN patients; 152 neuroendocrine carcinomas (NEC) and 29 neuroendocrine tumours (NET G3). Based on the sequencing of 360 cancer-related genes, we assessed mutatio
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Karabela, Sophia, Ioannis Psallidas, Taylor Sherrill та ін. "Opposing effects of bortezomib-induced nuclear factor-κB inhibition on chemical lung carcinogenesis." Carcinogenesis 33, № 4 (2012): 859–67. https://doi.org/10.1093/carcin/bgs024.

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Since recent evidence indicates a requirement for epithelial nuclear factor (NF)-κB signaling in lung tumorigenesis, we investigated the impact of the NF-κB inhibitor bortezomib on lung tumor promotion and growth. We used an experimental model in which wild-type mice or mice expressing an NF-κB reporter received intraperitoneal urethane (1 g/kg) followed by twice weekly bortezomib (1 mg/kg) during distinct periods of tumor initiation/progression. Mice were serially assessed for lung NF-κB activation, inflammation and carcinogenesis. Short-term proteasome inhibition with bortezomib did not impa
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Mikityuk, Myroslava, Oksana Khizhnyak, O. V. Tyazhelova, I. І. Alekseeva, and G. G. Kravchenko. "A CASE OF ACTIVE ACROMEGALIA COMPLICATED KIDNEY CANCER WITH ADNASTAL METASTASIS." Problems of Endocrine Pathology 32, no. 2 (2010): 59–65. http://dx.doi.org/10.21856/j-pep.2010.2.09.

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Insulin-like growth factor-1 (IRF-1) plays an important role in the development of malignant neoplasms such as colorectal cancer, breast and lung cancer. Several clinical and experimental studies have demonstrated the effect of high levels of IRF-1 in the blood and the expression of its receptors in tissues on their malignancy. Somatotropic hormone of the pituitary gland (STH) is involved in oncogenesis not only indirectly, by increasing the production of IRF-1 by the liver, but also directly but, realizing its effects through STG receptors (rSTG), are expressed in various tumors
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Grillo, Federica, Tullio Florio, Francesco Ferraù, et al. "Emerging multitarget tyrosine kinase inhibitors in the treatment of neuroendocrine neoplasms." Endocrine-Related Cancer 25, no. 9 (2018): R453—R466. http://dx.doi.org/10.1530/erc-17-0531.

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In the last few years, the therapeutic approach for neuroendocrine neoplasms (NENs) has changed dramatically following the approval of several novel targeted treatments. The multitarget tyrosine kinase inhibitor (MTKI), sunitinib malate, has been approved by Regulatory Agencies in pancreatic NENs. The MTKI class, however, includes several other molecules (approved for other conditions), which are currently being studied in NENs. An in-depth review on the studies published on the MTKIs in neuroendocrine tumors such as axitinib, cabozantinib, famitinib, lenvatinib, nintedanib, pazopanib, sorafen
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Matar, Somer, Anna Malczewska, Kjell Oberg, et al. "Blood Chromogranin A Is Not Effective as a Biomarker for Diagnosis or Management of Bronchopulmonary Neuroendocrine Tumors/Neoplasms." Neuroendocrinology 110, no. 3-4 (2019): 185–97. http://dx.doi.org/10.1159/000500202.

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Background: Identification of circulating tumor markers for clinical management in bronchopulmonary (BP) neuroendocrine tumors/neoplasms (NET/NEN) is of considerable clinical interest. Chromogranin A (CgA), a “universal” NET biomarker, is considered controversial as a circulating biomarker of BPNEN. Aim: Assess utility of CgA in the diagnosis and management of BPNEN in a multicentric study. Material and Methods: CgA diagnostic metrics were assessed in lung NET/NENs (n = 200) and controls (n = 140), randomly assigned to a Training and Test set (100 BPC and 70 controls in each). Assay specificit
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28

Espinosa-de-los-Monteros, Ana Laura, Claudia Ramírez-Rentería, and Moisés Mercado. "CLINICAL HETEROGENEITY OF ECTOPIC ACTH SYNDROME: A LONG-TERM FOLLOW-UP STUDY." Endocrine Practice 26, no. 12 (2020): 1435–41. http://dx.doi.org/10.4158/ep-2020-0368.

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Objective: Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a heterogeneous condition caused by neuroendocrine neoplasms (NENs) located in the lungs, thymus, or pancreas. Our purpose was to evaluate the long-term outcome of these patients. Methods: Retrospective study at a referral center. The charts of 164 patients with Cushing syndrome, followed at our center from 1993 to 2019, were analyzed. Results: EAS was found in 16 patients (9.75%, 9 women, mean age 36.01 years) who had been followed for a median of 72 months. The source of EAS was a NEN in 10 patients (8 bronchial and 2 th
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Casey, Ruth T., Gerlof D. Valk, Camilla Schalin-Jäntti, Ashley B. Grossman, and Rajesh V. Thakker. "ENDOCRINOLOGY IN THE TIME OF COVID-19: Clinical management of neuroendocrine neoplasms (NENs)." European Journal of Endocrinology 183, no. 2 (2020): G79—G88. http://dx.doi.org/10.1530/eje-20-0424.

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In viral pandemics, most specifically Covid-19, many patients with neuroendocrine neoplasms (NENs), including phaeochromocytomas, paragangliomas and medullary thyroid carcinoma, may develop Covid-19 in a mild or severe form, or be concerned about the influence of viral infection relative to their anti-tumoral therapy. In general, newly presenting patients should be assessed, and patients recently receiving chemotherapy, targeted therapy or radionuclide therapy, or showing tumour growth, should be closely followed. For previously diagnosed patients, who have indolent disease, some delay in rout
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Albertelli, Manuela, Andrea Dotto, Federica Nista, et al. ""Present and future of immunotherapy in Neuroendocrine Tumors"." Reviews in Endocrine and Metabolic Disorders 22, no. 3 (2021): 615–36. http://dx.doi.org/10.1007/s11154-021-09647-z.

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AbstractImmunotherapy, so promising in many neoplasms, still does not have a precise role in the treatment of neuroendocrine neoplasms (NENs). In this article, we provide an overview on the current knowledge about immunotherapy with immune checkpoint inhibitors (ICIs) applied to NENs, evaluating future perspectives in this setting of tumors.Evidence so far available for ICIs in gastroenteropancreatic (GEP)-NENs is definitively not as robust as for other tumors such as Small Cell Lung Cancer or Merkel Cell Carcinoma. In fact, with regard to the well-differentiated forms of NENs (NETs), the resu
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Rindi, G., C. Klersy, F. Inzani, et al. "Grading the neuroendocrine tumors of the lung: an evidence-based proposal." Endocrine-Related Cancer 21, no. 1 (2014): 1–16. http://dx.doi.org/10.1530/erc-13-0246.

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Lung neuroendocrine tumors are catalogued in four categories by the World Health Organization (WHO 2004) classification. Its reproducibility and prognostic efficacy was disputed. The WHO 2010 classification of digestive neuroendocrine neoplasms is based on Ki67 proliferation assessment and proved prognostically effective. This study aims at comparing these two classifications and at defining a prognostic grading system for lung neuroendocrine tumors. The study included 399 patients who underwent surgery and with at least 1 year follow-up between 1989 and 2011. Data on 21 variables were collect
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Dong, Qihan, Larisa V. Debelenko, Settara C. Chandrasekharappa, et al. "Loss of Heterozygosity at 11q13: Analysis of Pituitary Tumors, Lung Carcinoids, Lipomas, and Other Uncommon Tumors in Subjects with Familial Multiple Endocrine Neoplasia Type 1." Journal of Clinical Endocrinology & Metabolism 82, no. 5 (1997): 1416–20. http://dx.doi.org/10.1210/jcem.82.5.3944.

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Abstract Loss of heterozygosity (LOH) for polymorphic markers flanking the multiple endocrine neoplasia type 1 (MEN-1) gene in parathyroid and pancreatic islet tumors from subjects with familial MEN-1 (FMEN-1) has been well documented and has led to the hypothesis that the MEN-1 gene functions as a tumor suppressor. To assess the role of the MEN-1 gene in the pathogenesis of tumors less commonly associated with MEN-1, we employed a large number of highly informative polymorphic markers closely linked to the MEN-1 gene to study a series of 13 such tumors from subjects with FMEN-1 for LOH at 11q
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Chatzellis, Eleftherios, Anna Angelousi, Kosmas Daskalakis, et al. "Activity and Safety of Standard and Prolonged Capecitabine/Temozolomide Administration in Patients with Advanced Neuroendocrine Neoplasms." Neuroendocrinology 109, no. 4 (2019): 333–45. http://dx.doi.org/10.1159/000500135.

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Background: Capecitabine and temozolomide combination (CAPTEM) is associated with high response rates in patients with advanced neuroendocrine neoplasms (NENs). We evaluated the real-world activity and safety of CAPTEM from 3 NEN centers. Methods: Clinicopathological characteristics and outcomes of patients treated with CAPTEM for bulky or progressive disease (PD) were retrospectively analyzed. ­Results: Seventy-nine patients with gastroenteropancreatic (grades 1–2 [n = 38], grade 3 [n = 24]) and lung/thymic (n = 17) NENs were included. Median treatment duration was 12.1 months (range 0.6–55.6
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Lim, Kok Haw Jonathan, Hussain Raja, Paolo D’Arienzo, et al. "Identification of Areas for Improvement in the Management of Bone Metastases in Patients with Neuroendocrine Neoplasms." Neuroendocrinology 110, no. 7-8 (2019): 688–96. http://dx.doi.org/10.1159/000504256.

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Background: There is no global consensus on the optimal management of bone metastases (BMs) in neuroendocrine neoplasms (NENs). Objectives: To review current management and outcomes of patients with BMs in NENs, in order to identify areas for improvement. Methods: A retrospective study of all patients with NENs, except Grade 3 lung NENs (April 2002 to March 2018) was conducted. Baseline characteristics, nature of BMs, treatment received and overall survival (OS) were evaluated. Statistical analyses were performed using SPSS version 23.0/STATA v12. Results: Of 1,212 patients, 85 (7%) had BMs; m
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Kozlov, Sergey S., Larisa A. Ermakova, Yuri V. Lobzin, et al. "A case of pleuropulmonary dirofilariasis caused by Dirofilaria repens. Case report." Terapevticheskii arkhiv 95, no. 11 (2023): 970–75. http://dx.doi.org/10.26442/00403660.2023.11.202477.

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The differential diagnosis of neoplasms of various localizations is the primary task in clinical practice of all physicians. We present a description of the case of invasion with Dirofilaria repens in the lung of a 68-year-old patient. In 2016 the patient was diagnosed with cancer of the left kidney and underwent a left-sided nephrectomy. During the dynamic observation in 2019, a lump was found in the left lung, which was regarded as a metastasis. An atypical SIX resection of the left lung was performed. Microscopy of the removed lump revealed the presence of a nematode of the genus Dirofilari
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Srivastava, Ritesh K., Changzhao Li, Jasim Khan, Nilam Sanjib Banerjee, Louise T. Chow, and Mohammad Athar. "Combined mTORC1/mTORC2 inhibition blocks growth and induces catastrophic macropinocytosis in cancer cells." Proceedings of the National Academy of Sciences 116, no. 49 (2019): 24583–92. http://dx.doi.org/10.1073/pnas.1911393116.

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The mammalian target of rapamycin (mTOR) pathway, which plays a critical role in regulating cellular growth and metabolism, is aberrantly regulated in the pathogenesis of a variety of neoplasms. Here we demonstrate that dual mTORC1/mTORC2 inhibitors OSI-027 and PP242 cause catastrophic macropinocytosis in rhabdomyosarcoma (RMS) cells and cancers of the skin, breast, lung, and cervix, whereas the effects are much less pronounced in immortalized human keratinocytes. Using RMS as a model, we characterize in detail the mechanism of macropinocytosis induction. Macropinosomes are distinct from endoc
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Wurth, Rachel, Abhishek Jha, Crystal Kamilaris, et al. "A Case of Carney Triad Complicated by Renal Cell Carcinoma and a Germline SDHA Pathogenic Variant." Journal of the Endocrine Society 5, Supplement_1 (2021): A985. http://dx.doi.org/10.1210/jendso/bvab048.2015.

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Abstract Background: Carney triad is a rare multiple-neoplasia syndrome presenting as an association of paragangliomas (PGL), gastrointestinal stromal tumors (GIST), and pulmonary chondromas (CHO). Succinate dehydrogenase deficiency has been associated with several neoplasias, including Carney triad, renal cell carcinoma (RCC) and those associated with hereditary PGL/ pheochromocytoma (PHEO) syndromes. Clinical Case: A 57-year-old male diagnosed with hypertension at age 49, presented with a gradual increase in blood pressure over a period of 12 months. For seven years following his diagnosis o
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Mon Zin, Myet, Sriprakash Mokshagundam, and Jonathan Cunningham. "ODP267 Doege-Potter Syndrome in a Patient With Mediastinal Solitary Fibrous Tumor." Journal of the Endocrine Society 6, Supplement_1 (2022): A432. http://dx.doi.org/10.1210/jendso/bvac150.898.

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Abstract N on-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndromethat presents mostly with hypoinsulinemic hypoglycemia and is associated with benign and malignant neoplasms other than insulinoma. Doege-Potter Syndrome (DPS) is characterized when NICTH is secondary to a solitary fibrous tumor (SFT). The following is a case of DPS in which the patient presented with refractory hypoglycemia. An 81-year-old Caucasian male presented to emergency department with a 10-day history of worseningneuroglycopenic symptoms in fasting state. He experienced worsening night sweats, unstead
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Polymeris, Antonis, Christina Kogia, Dimitrios Ioannidis, et al. "Excessive Leukocytosis Leading to a Diagnosis of Aggressive Thyroid Anaplastic Carcinoma: A Case Report and Relevant Review." European Thyroid Journal 9, no. 3 (2020): 162–68. http://dx.doi.org/10.1159/000506767.

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Introduction: Leukocytosis and particularly neutrophilia are usually caused by acute infection, inflammation, and myeloproliferative neoplasms. However, leukocytosis can also occur in patients with malignancy either due to bone marrow metastases or in the context of a paraneoplastic syndrome. Case Presentation: An 86-year-old female was admitted to our hospital due to marked leukocytosis (white blood cells [WBC] >40,000/μL), neutrophilia, and monocytosis. She was afebrile and reported hoarseness and mild difficulty swallowing. Upon physical examination, lung auscultation revealed inspirator
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Pereira, Esdras E. B., Antônio A. C. Modesto, Bruno M. Fernandes, et al. "Association between Polymorphism of Genes IL-1A, NFKB1, PAR1, TP53, and UCP2 and Susceptibility to Non-Small Cell Lung Cancer in the Brazilian Amazon." Genes 14, no. 2 (2023): 461. http://dx.doi.org/10.3390/genes14020461.

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Non-small cell lung cancer (NSCLC) accounts for the vast majority of cases of lung neoplasms. It is formed in multiple stages, with interactions between environmental risk factors and individual genetic susceptibility and with genes involved in the immune and inflammatory response paths, cell or genome stability, and metabolism, among others. Our objective was to evaluate the association between five genetic variants (IL-1A, NFKB1, PAR1, TP53, and UCP2) and the development of NSCLC in the Brazilian Amazon. The study included 263 individuals with and without lung cancer. The samples were analyz
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Gohil, Shailesh, Karen Page, Hastings Rob, et al. "RF19 | PSUN353 Liquid Biopsies - Detecting and Tracking Circulating Cell Free Tumor Derived DNA in Patients with Neuroendocrine Neoplasms." Journal of the Endocrine Society 6, Supplement_1 (2022): A898—A899. http://dx.doi.org/10.1210/jendso/bvac150.1861.

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Abstract Introduction There is a clinical need to develop novel and better biomarkers to monitor patients with neuroendocrine neoplasms (NENs). Circulating cell free tumor derived DNA (ctDNA), a form of liquid biopsy, is finding clinical utility in an ever increasing number of malignancies however has not been widely tested in patients with NENs. Aims Our aim was to identify and track plasma ctDNA in a cohort of patients with NENs using a personalized, patient specific approach. Materials and methods A total of 35 serial plasma samples were collected from 9 patients with metastatic, well diffe
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Kasajima, Atsuko, Björn Konukiewitz, Naomi Oka, et al. "Clinicopathological Profiling of Lung Carcinoids with a Ki67 Index > 20%." Neuroendocrinology 108, no. 2 (2018): 109–20. http://dx.doi.org/10.1159/000495806.

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The clinicopathological features of lung neuroendocrine neoplasms (NEN) with a high proliferative index at the border area between atypical carcinoid and neuroendocrine carcinoma have not been investigated so far. The aim of this study was, therefore, to search for lung NENs which are well differentiated but show Ki67 values that overlap with those of poorly differentiated (PD)-NENs. Resected lung NENs from 244 Japanese patients were reviewed, and Ki67 indices were assessed in all tumors. The data were then correlated to clinicopathological parameters and patient outcome. Among 59 (24%) well-d
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Daskalakis, Kosmas, Marina Tsoli, Raj Srirajaskanthan, et al. "Lung Metastases in Patients with Well-Differentiated Gastroenteropancreatic Neuroendocrine Neoplasms: An Appraisal of the Validity of Thoracic Imaging Surveillance." Neuroendocrinology 108, no. 4 (2019): 308–16. http://dx.doi.org/10.1159/000497183.

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Background/Aims: To evaluate the impact of lung metastases (LM) on overall survival (OS) in well-differentiated (WD) stage IV gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN) patients along with developing surveillance strategies for thoracic imaging. Methods: Thirty-four patients with LM, from 3 centres, were identified (22 small intestine/12 pancreatic; 17 grade 1/15 grade 2/2 of unknown grade). For comparison, we used 106 stage IV WD, grade 1 and 2 GEP-NEN patients with metastatic disease confined in the abdomen. Results: LM prevalence was 4.9% (34/692). Eleven patients (32%) prese
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Fénichel, Patrick, Cécile Rouzier, Catherine Butori та ін. "Extragestational βHCG Secretion Due to an Isolated Lung Epithelioid Trophoblastic Tumor: Microsatellite Genotyping of Tumoral Cells Confirmed Their Placental Origin and Oriented Specific Chemotherapy". Journal of Clinical Endocrinology & Metabolism 99, № 10 (2014): 3515–20. http://dx.doi.org/10.1210/jc.2014-1460.

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Abstract Context: Persistent secretion of β-human chorionic gonadotropin (βHCG) in the absence of an ongoing or recent pregnancy and without persistent uterine gestational disease is a rare but challenging situation that requires locating the extrauterine secreting tumor and distinguishing between extragestational choriocarcinoma and gestational trophoblastic neoplasms. Case Presentation: An unexplained, persistent extragestational βHCG secretion occurring in a 29-year-old, nonsmoking woman with abnormal uterine bleeding 4 years after a normal pregnancy and without persistent gestational disea
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Rösner, Erik, Daniel Kaemmerer, Elisa Neubauer, Jörg Sänger, and Amelie Lupp. "Prognostic value of PD-L1 expression in bronchopulmonary neuroendocrine tumours." Endocrine Connections 10, no. 2 (2021): 180–90. http://dx.doi.org/10.1530/ec-20-0540.

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Programmed death protein 1 (PD-1) and its ligand, PD-L1, have emerged as promising therapeutic targets for many types of cancer that overexpress PD-L1. However, data on PD-L1 expression levels in bronchopulmonary neuroendocrine neoplasms (BP-NEN) are limited and contradictory. In the present study, a total of 298 archived, formalin-fixed, paraffin-embedded BP-NEN samples from 97 patients diagnosed with typical carcinoid (TC), atypical carcinoid (AC), small cell lung cancer (SCLC), or large cell neuroendocrine carcinoma of the lung (LCNEC) were evaluated for PD-L1 expression by immunohistochemi
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Sahdev, Anju, Rodney H. Reznek, Jane Evanson, and Ashley B. Grossman. "Imaging in Cushing's syndrome." Arquivos Brasileiros de Endocrinologia & Metabologia 51, no. 8 (2007): 1319–28. http://dx.doi.org/10.1590/s0004-27302007000800018.

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Once the diagnosis of Cushing's syndrome (CS) has been established, the main step is to differentiate between ACTH dependent and independent disease. In adults, 80% of CS is due to ACTH-dependent causes and 20% due to adrenal causes. ACTH-secreting neoplasms cause ACTH-dependent CS. These are usually anterior pituitary microadenomas, which result in the classic Cushing's disease. Non-pituitary ectopic sources of ACTH, such as a small-cell lung carcinoma or carcinoid tumours, are the source of the remainder of ACTH-dependent disease. In the majority of patients presenting with clinical and bioc
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Godlewska, Magdalena, Andrzej Jerzy Nowak, Anna Boguslawska, et al. "Diverse Manifestation of Acromegaly With Suspicion of Ectopic GHRH Secretion. Report of Two Cases." Journal of the Endocrine Society 5, Supplement_1 (2021): A582—A583. http://dx.doi.org/10.1210/jendso/bvab048.1188.

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Abstract Introduction: Acromegaly can rarely be caused by an ectopic production of GH or GHRH by various neoplasms, most commonly neuroendocrine tumors of pancreatic or pulmonary origin. Squamous cell carcinoma of the lung has not been associated with ectopic GHRH secretion yet. The authors present two cases of acromegaly with suspicion of GHRH ectopy. Presentation of the Cases: Case 1. A male born in 1945 presenting with typical morphologic features of acromegaly. Pituitary MRI revealed a cuneatic-shaped, hypointense focal lesion (8x7x6 mm) in the right posterior part of the anterior pituitar
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Priyadarshini, Shista, Bikal Lamichhane, and F. N. U. Manas. "LBMON138 Leg Tumeo: Is It Neuroendocrine Tumor?" Journal of the Endocrine Society 6, Supplement_1 (2022): A468—A469. http://dx.doi.org/10.1210/jendso/bvac150.974.

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Abstract Introduction Neuroendocrine tumors (NETs) are heterogenous group of epithelial neoplasms often known to produce and secrete peptides and bioamines. The presentation varies from symptomatic functional to non-functional tumors. The functional tumors cause symptoms based on the substance secreted: corticotropin, gastrin, glucagon, insulin, norepinephrine, serotonin, somatostatin, etc. The prognosis ranges from well-differentiated tumors being indolent while the poorly differentiated forms are aggressive. We present a case with an atypical presentation of NET which was noted to secrete an
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Bajetta, Emilio, Laura Catena, Sara Pusceddu, et al. "Everolimus in Combination with Octreotide Long-Acting Repeatable in a First-Line Setting for Patients with Neuroendocrine Tumors: A 5-Year Update." Neuroendocrinology 106, no. 4 (2017): 307–11. http://dx.doi.org/10.1159/000479587.

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Background: We previously presented data of this multicentric, phase II study showing that everolimus plus octreotide long-acting repeatable (LAR) for advanced neuroendocrine neoplasms (NENs), in the first line setting, is an active and safe treatment. We now present updated data at 5 years. Methods: Patients with advanced well-differentiated, previously untreated neuroendocrine tumors of the gastroenteropancreatic tract and of the lung received octreotide LAR 30 mg plus everolimus 10 mg/day. The primary endpoint was the objective response rate (ORR). We performed an analysis of “long responde
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Guseinova, R. M., E. A. Pigarova, L. K. Dzeranova, M. S. Sheremeta, and E. G. Przhiyalkovskaya. "Treatment of ACTH-ectopic syndrome with long-acting octreotide: effective control of disease activity." Obesity and metabolism 19, no. 2 (2022): 180–88. http://dx.doi.org/10.14341/omet12876.

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ACTH — ectopic syndrome (ACTH-ES) is a severe multisystem disease caused by paraneoplastic secretion of ACTH itself and/or much less often corticoliberin (CL) by tumor tissue. The frequency of ACTH-ES is 12–20% of cases of endogenous hypercortisolism, i.e. about 1–2 cases per million population, and covers a range of tumors, from benign neoplasms to malignant tumors with widespread metastases, while the most common causes of ACTH-ES are tumors of the lung, pancreas and thymus, and more rare localizations are neuroendocrine tumors (NET) of the intestine, medullary thyroid cancer, pheochromocyto
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