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J Thorac Oncol. 2014 Aug;9(8):1162-70. doi: 10.1097/JTO.0000000000000209.

Lung cancer in combined pulmonary fibrosis and emphysema: a series of 47 Western patients.

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*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.



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.


This retrospective multicentre study was conducted by the Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).


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.


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.

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