J Thorac Oncol 2014 Aug;9(8):1162-70
*Department of Respiratory Medicine, National Reference Center for rare pulmonary diseases, Hôpital Louis Pradel, Hospices Civils de Lyon; Université Claude Bernard Lyon 1, Lyon; †Department of Respiratory Medicine, CHRU Tours, Université François Rabelais, INSERM UMR 1100, Tours; ‡Chest Department, Expert Center of Thoracic Oncology, Regional Reference Center for rare pulmonary diseases, Hôpital Tenon, Assistance Publique Hôpitaux de Paris; Université Pierre et Marie Curie; §Department of Respiratory Medicine, Competence Center for Rare Pulmonary Diseases, Hôpital Bichat, Assistance Publique Hôpitaux de Paris, Paris; ‖Department of Respiratory Medicine, CHU Angers, Angers; ¶Department of Respiratory Medicine, Competence Center for rare pulmonary diseases, Hôpital Pontchaillou, IRSET UMR 1085, Université de Rennes 1, Rennes, France; #Department of Respiratory Medicine, CHU Mont-Godine, Université Catholique de Louvain, Yvoir, Belgium; and **Pulmonology Service, Hôpital de Bourgoin-Jallieu, Bourgoin-Jallieu, France.
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.