Lung cancer in combined pulmonary fibrosis and emphysema: A series of 47 western patients

Nicolas Girard, Sylvain Marchand-Adam, Jean Marc Naccache, Raphael Borie, Thierry Urban, Stéphane Jouneau, Eric Marchand, Anne Claire Ravel, Lize Kiakouama, Bénédicte Etienne-Mastroianni, Jacques Cadranel, Vincent Cottin, Jean François Cordier

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)1162-1170
Number of pages9
JournalJournal of Thoracic Oncology
Volume9
Issue number8
DOIs
Publication statusPublished - 2014
Externally publishedYes

Keywords

  • Combined pulmonary fibrosis and emphysema syndrome
  • Lung cancer
  • Orphan disease. Tobacco smoking

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