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Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids

2015; Elsevier BV; Volume: 26; Issue: 8 Linguagem: Inglês

10.1093/annonc/mdv041

1569-8041

M. Caplin, Éric Baudin, Piero Ferollà, Pier Luigi Filosso, Mariano García‐Yuste, Eric Lim, Kjell Öberg, Giuseppe Pelosi, Aurel Perren, Roberta Rossi, William D. Travis, D.K. Bartsch, Jaume Capdevila, Frederico Costa, Jarosław B. Ćwikła, Wouter W. de Herder, Gianfranco Delle Fave, Barbro Eriksson, Massimo Falconi, Diego Ferone, David J. Gross, Ashley Grossman, Tetsuhide Ito, Robert T. Jensen, Gregory Kaltsas, Fahrettin Keleştimur, Réza Kianmanesh, Ulrich Knigge, Beata Kos‐Kudła, Eric P. Krenning, Emmanuel Mitry, M. Nicolson, Juan Manuel O’Connor, Dermot O’Toole, Ulrich-Frank Pape, Marianne Pavel, John Ramage, Éric Raymond, Guido Rindi, Andrea Rockall, Philippe Ruszniewski, Ramón Salazar, Aldo Scarpa, Eva Sedláčková, Anders Sundin, Christos Toumpanakis, Marie‐Pierre Vullierme, Wolfgang Weber, Bertram Wiedenmann, Zeng Zheng-Pei,

Lung Cancer Research Studies

BackgroundPulmonary 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 methodsBibliographical 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.ResultsPCs 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.ConclusionsPCs are complex tumors which require a multidisciplinary approach and long-term follow-up.