Recent Advances in Chest Medicine |

Advances in the Diagnosis and Management of Well-Differentiated and Intermediate-Differentiated Neuroendocrine Tumors of the Lung OPEN ACCESS

Edward M. Wolin, MD
Author and Funding Information

Conflict of Interest: E.W. has received consulting fees from Novartis, Ipsen, Celgene, and Advanced Accelerator Applications.

Funding Information: Financial support for editorial assistance was provided by Novartis Pharmaceuticals, Inc., in compliance with international guidelines on Good Publication Practice.

From Montefiore M-E Center for Cancer Care, Bronx, NY

Correspondence to: Edward M. Wolin, MD, Montefiore M-E Center for Cancer Care, 1521 Jarrett Place, Bronx, NY 10461-2601.

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016. doi:10.1016/j.chest.2016.06.018
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Neuroendocrine tumors (NET) are a rare, heterogeneous group of malignancies that arise from neuroendocrine cells throughout the body, with the lungs and gastrointestinal tract being the most common sites of origin. Despite increasing incidence, awareness of lung NET remains low among thoracic specialists who are often involved in assessment and early treatment for these patients. Successful treatment requires accurate and timely diagnosis; however, classification can be challenging, particularly for well-differentiated and intermediate-differentiated lung NET types (typical carcinoids [TC] and atypical carcinoids [AC]). Diagnosis and management of lung NET are further complicated by the nonspecificity of symptoms, variable natural history, and lack of high-level clinical evidence requiring a multidisciplinary approach, which has been shown to improve prognosis. Currently, surgery remains the only curative option for TC/AC. Inconsistencies between guideline recommendations for systemic therapies, especially radiation and chemotherapy, result in a lack of consensus on a standardized treatment for unresectable disease. Recent data from the phase 3 RADIANT-4 trial, which contained a large population of patients with advanced, well-differentiated, nonfunctional lung NET in addition to those with gastrointestinal NET, showed a reduced risk of disease progression and death with everolimus compared with placebo, leading to US approval of everolimus in these patient populations. This is the first high-level therapeutic evidence in patients with TC/AC, and everolimus is currently the only agent approved for TC/AC treatment. Increased awareness, prompt diagnosis, and additional adequately powered controlled clinical trials of patients with well-differentiated and intermediate-differentiated lung NET are needed to further improve evidence-based care.

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