@article{3109970, title = "Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids", author = "Caplin, M.E. and Baudin, E. and Ferolla, P. and Filosso, P. and Garcia-Yuste, M. and Lim, E. and Oberg, K. and Pelosi, G. and Perren, A. and Rossi, R.E. and Travis, W.D. and Bartsch, D. and Capdevila, J. and Costa, F. and Cwikla, J. and Herder, W. and Fave, G.D. and Eriksson, B. and Falconi, M. and Ferone, D. and Gross, D. and Grossman, A. and Ito, T. and Jensen, R. and Kaltsas, G. and Kelestimur, F. and Kianmanesh, R. and Knigge, U. and Kos-Kudla, B. and Krenning, E. and Mitry, E. and Nicolson, M. and O'Connor, J. and O'Toole, D. and Pape, U.-F. and Pavel, M. and Ramage, J. and Raymond, E. and Rindi, G. and Rockall, A. and Ruszniewski, P. and Salazar, R. and Scarpa, A. and Sedlackova, E. and Sundin, A. and Toumpanakis, C. and Vullierme, M.-P. and Weber, W. and Wiedenmann, B. and Zheng-Pei, Z. and ENETS consensus conference participants", journal = "Annals of Oncology", year = "2015", volume = "26", number = "8", pages = "1604-1620", publisher = "Oxford University Press", issn = "0923-7534, 1569-8041", doi = "10.1093/annonc/mdv041", keywords = "bevacizumab; chromogranin A; cisplatin; corticotropin; cytokeratin; dacarbazine; erlotinib; etomidate; everolimus; fluorouracil; growth hormone releasing factor; ketoconazole; Ki 67 antigen; metyrapone; mifepristone; octreotide; octreotide tetraxetan lu 177; octreotide tetraxetan y 90; pazopanib; pentetreotide; placebo; radiopharmaceutical agent; somatomedin C; somatostatin derivative; somatostatin receptor; sorafenib; streptozocin; temozolomide; thyroid transcription factor 1; unclassified drug; unindexed drug; antineoplastic agent; carboplatin; cisplatin; dacarbazine; etoposide; somatostatin receptor; temozolomide, Article; bronchoscopy; cancer combination chemotherapy; cancer epidemiology; cancer grading; cancer palliative therapy; cancer radiotherapy; cancer staging; carcinoid; clinical practice; computer assisted tomography; contrast radiography; Cushing syndrome; echocardiography; follow up; histopathology; human; immunohistochemistry; immunoreactivity; liver metastasis; liver resection; lung biopsy; lung function test; lung resection; metastasis resection; multiple endocrine neoplasia type 1; neuroendocrine tumor; nuclear magnetic resonance imaging; phase 2 clinical trial (topic); phase 3 clinical trial (topic); priority journal; pulmonary carcinoid; systematic review; thorax radiography; tumor differentiation; analogs and derivatives; carcinoid syndrome; Carcinoid Tumor; consensus development; echography; Europe; Lung Neoplasms; medical society; metabolism; positron emission tomography; practice guideline, Antineoplastic Combined Chemotherapy Protocols; Bronchoscopy; Carboplatin; Carcinoid Heart Disease; Carcinoid Tumor; Cisplatin; Dacarbazine; Etoposide; Europe; Humans; Lung Neoplasms; Pneumonectomy; Positron-Emission Tomography; Receptors, Somatostatin; Societies, Medical; Tomography, X-Ray Computed", abstract = "Background: Pulmonary carcinoids (PCs) are rare tumors. As there is a paucity of randomized studies, this expert consensus document represents an initiative by the European Neuroendocrine Tumor Society to provide guidance on their management. Patients and methods: Bibliographical searches were carried out in PubMed for the terms 'pulmonary neuroendocrine tumors', 'bronchial neuroendocrine tumors', 'bronchial carcinoid tumors', 'pulmonary carcinoid', 'pulmonary typical/atypical carcinoid', and 'pulmonary carcinoid and diagnosis/treatment/epidemiology/prognosis'. A systematic review of the relevant literature was carried out, followed by expert review. Results: PCs are well-differentiated neuroendocrine tumors and include low- and intermediate-grade malignant tumors, i.e. typical (TC) and atypical carcinoid (AC), respectively. Contrast CT scan is the diagnostic gold standard for PCs, but pathology examination is mandatory for their correct classification. Somatostatin receptor imaging may visualize nearly 80% of the primary tumors and is most sensitive for metastatic disease. Plasma chromogranin A can be increased in PCs. Surgery is the treatment of choice for PCs with the aim of removing the tumor and preserving as much lung tissue as possible. Resection of metastases should be considered whenever possible with curative intent. Somatostatin analogs are the first-line treatment of carcinoid syndrome and may be considered as first-line systemic antiproliferative treatment in unresectable PCs, particularly of low-grade TC and AC. Locoregional or radiotargeted therapies should be considered for metastatic disease. Systemic chemotherapy is used for progressive PCs, although cytotoxic regimens have demonstrated limited effects with etoposide and platinum combination the most commonly used, however, temozolomide has shown most clinical benefit. Conclusions: PCs are complex tumors which require a multidisciplinary approach and long-term follow-up. © The Author 2015. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved." }