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Neuroendocrine tumors (NETs) of the gastrointestinal tract and lungs are rare yet clinically significant neoplasms characterized by heterogeneous progression and diverse manifestations. This article systematizes current management strategies based on the latest recommendations and scientific advances. Epidemiological trends are a key focus, including an increase in NET incidence rates over the past few decades (6.4 times in the USA from 1973 to 2012) and the predominance of gastroenteropancreatic NETs (62–70%) and bronchopulmonary NETs (25%). The following key clinical aspects are highlighted, including hormonally active conditions (carcinoid syndrome, gastrinomas, insulinomas) and their complications (carcinoid heart disease and crises). Current approaches to diagnose, treatment, and monitoring of NETs are discussed using recent guidelines and scientific data. Molecular genetic testing is emphasized because of its ability to improve risk stratification and personalize treatment. The following treatment options are discussed: surgery (resection, liver transplantation); pharmacotherapy (somatostatin analogues, telotristat, and targeted therapies such as sunitinib and everolimus); chemotherapy; peptide receptor radionuclide therapy. The key study outcomes are presented. The article also addresses challenges in early diagnosis and the need for a multidisciplinary approach and personalized treatment. Promising areas of using novel biomarkers and imaging techniques are mentioned, and the importance of follow-up is emphasized, including follow-up intervals and watch and wait strategies for small tumors. The article discusses the challenges of early diagnosis and the importance of thorough tumor evaluations and explores prospects for further research to optimize treatment strategies.
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Mikheeva et al. (2025) studied this question.
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