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

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


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.

Publication types

  • Multicenter Study

MeSH terms

  • Aged
  • Antineoplastic Agents, Hormonal / therapeutic use
  • Dyspnea / etiology
  • Emphysema / diagnostic imaging*
  • Emphysema / physiopathology
  • Female
  • Forced Expiratory Volume
  • France
  • Humans
  • Lung Neoplasms / diagnosis
  • Lung Neoplasms / pathology*
  • Lung Neoplasms / therapy*
  • Male
  • Middle Aged
  • Pneumonectomy
  • Pulmonary Fibrosis / diagnostic imaging*
  • Pulmonary Fibrosis / physiopathology
  • Pulmonary Gas Exchange
  • Radiotherapy, Adjuvant
  • Retrospective Studies
  • Smoking
  • Survival Rate
  • Syndrome
  • Tomography, X-Ray Computed
  • Vital Capacity


  • Antineoplastic Agents, Hormonal