Abstract
NENs are a heterogeneous family of tumors of challenging diagnosis and clinical management. Their incidence and prevalence continue to rise across all sites, stages and grades. Although improved diagnostic techniques have led to earlier detection and stage migration, the improved prognosis documented over time for advanced gastrointestinal and pancreatic neuroendocrine tumors also reflect improvements in therapy. The aim of this guideline is to update practical recommendations for the diagnosis and treatment of gastroenteropancreatic and lung NENs. Diagnostic procedures, histological classification and therapeutic options are briefly discussed, including surgery, liver-directed therapy, peptide receptor radionuclide therapy, and systemic hormonal, cytotoxic or targeted therapy, and treatment algorithms are provided.
Highlights
Diagnostic proceduresThe incidence of NENs has increased 6.4-fold over the past four decades, from 1.09 (1973) to 6.98 (2012) new cases per 100,000 inhabitants annually [1]
They are generally diagnosed in the fifth decade of life, and about 5% of them are associated with hereditary predisposition syndromes
We aim to provide synthetical and practical guidelines regarding diagnostic procedures and therapeutic options for the multidisciplinary management of NENs of GEP or lung origin
Summary
The incidence of NENs has increased 6.4-fold over the past four decades, from 1.09 (1973) to 6.98 (2012) new cases per 100,000 inhabitants annually [1]. About 20–25% of NENs are functioning tumors, that is, they are associated with a clinical syndrome due to excessive hormone production (carcinoid syndrome, Zollinger–Ellison syndrome, etc.). This proportion is decreasing with time due to earlier diagnosis and improved symptomatic and antineoplastic therapy [3]. NENs are a complex, heterogenous family of tumors of challenging clinical management. A thoracic CT scan may be considered in poorly differentiated tumors, colon primaries or those in whom surgery of liver metastasis is being considered and is mandatory in lung NENs. I. Small randomized trials or large randomized trials with a suspicion of bias (lower methodological quality) or metaanalyses of such trials or of trials with demonstrated heterogeneity
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