Advances in the diagnosis and management of well-differentiated and intermediate-differentiated neuroendocrine tumors of the lung
Cet article présente les avancées récentes concernant le diagnostic et la prise en charge des tumeurs pulmonaires neuroendocrines bien ou moyennement différenciées
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.
Chest , article en libre accès, 2015