title: Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids.
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.
  
  
  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.
authors: Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK m.caplin@ucl.ac.uk.
authors: M E
authors: ME
authors: Caplin
authors: Department of Nuclear Medicine, Endocrine Cancer and Interventional Radiology, Institut Gustave Roussy, Université Paris Sud, Villejuif Cedex, France.
authors: E
authors: E
authors: Baudin
authors: NET Center, Umbria Regional Cancer Network, Università degli Studi di Perugia, Perugia.
authors: P
authors: P
authors: Ferolla
authors: Department of Thoracic Surgery, University of Torino, Torino, Italy.
authors: P
authors: P
authors: Filosso
authors: Department of Thoracic Surgery, University Clinic Hospital, Valladolid, Spain.
authors: M
authors: M
authors: Garcia-Yuste
authors: Imperial College and The Academic Division of Thoracic Surgery, The Royal Brompton Hospital, London, UK.
authors: E
authors: E
authors: Lim
authors: Endocrine Oncology Unit, Department of Medicine, University Hospital, Uppsala, Sweden.
authors: K
authors: K
authors: Oberg
authors: Fondazione IRCCS Istituto Nazionale dei Tumori and Dipartimento di Scienze Biologiche e Cliniche Luigi Sacco, Università degli studi di Milano, Milan, Italy.
authors: G
authors: G
authors: Pelosi
authors: Institute of Pathology, University of Bern, Bern, Switzerland.
authors: A
authors: A
authors: Perren
authors: Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK Gastroenterology and Endoscopy Unit, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico and Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
authors: R E
authors: RE
authors: Rossi
authors: Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, USA.
authors: W D
authors: WD
authors: Travis
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001999:Bronchoscopy; D016190:Carboplatin / Q000008:administration & dosage; D002275:Carcinoid Heart Disease / Q000000981:diagnostic imaging; D002276:Carcinoid Tumor / Q000175:diagnosis / Q000628:therapy*; D002945:Cisplatin / Q000008:administration & dosage; D003606:Dacarbazine / Q000008:administration & dosage / Q000031:analogs & derivatives; D005047:Etoposide / Q000008:administration & dosage; D005060:Europe; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000628:therapy*; D011013:Pneumonectomy; D049268:Positron-Emission Tomography; D017481:Receptors, Somatostatin / Q000378:metabolism; D012955:Societies, Medical; D000077204:Temozolomide; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography
chemical_list: D017481:Receptors, Somatostatin; D005047:Etoposide; D003606:Dacarbazine; D016190:Carboplatin; D002945:Cisplatin; D000077204:Temozolomide
keywords: atypical carcinoid; bronchial; carcinoid; neuroendocrine tumor; pulmonary; typical carcinoid