TY - JOUR
T1 - Lung cancer in combined pulmonary fibrosis and emphysema
T2 - A series of 47 western patients
AU - Girard, Nicolas
AU - Marchand-Adam, Sylvain
AU - Naccache, Jean Marc
AU - Borie, Raphael
AU - Urban, Thierry
AU - Jouneau, Stéphane
AU - Marchand, Eric
AU - Ravel, Anne Claire
AU - Kiakouama, Lize
AU - Etienne-Mastroianni, Bénédicte
AU - Cadranel, Jacques
AU - Cottin, Vincent
AU - Cordier, Jean François
PY - 2014
Y1 - 2014
N2 - 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%), smallcell lung cancer in three (6%), and sarcomatoid carcinoma in one (2%). Overall, 20 of the 47 patients could not receive standard-ofcare 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.
AB - 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%), smallcell lung cancer in three (6%), and sarcomatoid carcinoma in one (2%). Overall, 20 of the 47 patients could not receive standard-ofcare 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.
KW - Combined pulmonary fibrosis and emphysema syndrome
KW - Lung cancer
KW - Orphan disease. Tobacco smoking
UR - http://www.scopus.com/inward/record.url?scp=84905833909&partnerID=8YFLogxK
U2 - 10.1097/JTO.0000000000000209
DO - 10.1097/JTO.0000000000000209
M3 - Article
C2 - 25157769
AN - SCOPUS:84905833909
SN - 1556-0864
VL - 9
SP - 1162
EP - 1170
JO - Journal of Thoracic Oncology
JF - Journal of Thoracic Oncology
IS - 8
ER -