Skip Navigation LinksHome > August 2014 - Volume 9 - Issue 8 > Lung Cancer in Combined Pulmonary Fibrosis and Emphysema: A...
Journal of Thoracic Oncology:
doi: 10.1097/JTO.0000000000000209
Original Articles

Lung Cancer in Combined Pulmonary Fibrosis and Emphysema: A Series of 47 Western Patients

Girard, Nicolas MD, PhD*; Marchand-Adam, Sylvain MD, PhD; Naccache, Jean-Marc MD; Borie, Raphael MD§; Urban, Thierry MD; Jouneau, Stéphane MD, PhD; Marchand, Eric MD, PhD#; Ravel, Anne-Claire MD**; Kiakouama, Lize MD*; Etienne-Mastroianni, Bénédicte MD*; Cadranel, Jacques MD, PhD; Cottin, Vincent MD, PhD*; Cordier, Jean-François MD*; for the Groupe d’Etudes et de Recherche sur les Maladies “Orphelines Pulmonaires (GERMOP)

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Introduction: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterized by imaging features consisting of the association of centrilobular and/or paraseptal emphysema and pulmonary fibrosis. Virtually all patients are smokers and thus at high risk of developing lung cancer.

Methods: This retrospective multicentre study was conducted by the Groupe d’Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM”O”P).

Results: A total of 47 patients presenting with lung cancer and CPFE syndrome were identified. All patients were smokers, with a mean of 47 pack-years. A pathological diagnosis of lung cancer was obtained for 38 (81%) patients. Histological type was squamous cell carcinoma in 17 (36%) patients, adenocarcinoma in 14 (30%), non–small-cell lung cancer not otherwise specified in three (6%), small-cell lung cancer in three (6%), and sarcomatoid carcinoma in one (2%). Overall, 20 of the 47 patients could not receive standard-of-care treatment for lung cancer, as per international recommendations or guidelines; this limitation was considered to be directly related to the CPFE syndrome in eight (40%) cases.

Conclusion: Lung cancer in patients with CPFE syndrome represents a specific entity with a poor prognosis, that further represents the most characteristic and severe model of tobacco-related disease.

Copyright © 2014 by the European Lung Cancer Conference and the International Association for the Study of Lung Cancer.


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