Author of

• 11946

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  P2.24 - Poster Session 2 - Supportive Care (ID 157)

  • Event: WCLC 2013
  • Type: Poster Session
  • Track: Supportive Care
  • Presentations: 1
  • Moderators:
  • Coordinates: 10/29/2013, 09:30 - 16:30, Exhibit Hall, Ground Level

  • 11984

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    P2.24-038 - Lung cancer and combined pulmonary fibrosis and emphysema syndrome in Western patients: a series of 47 patients (ID 2844)

    09:30 - 09:30  |  Author(s): E. Marchand
    • Abstract

    Background
    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, associated with subnormal spirometry, contrasting with severe impairment of gas exchange with strong decrease in carbon monoxide diffusing capacity, and hypoxaemia at exercise. Virtually all patients presenting with CPFE are smokers and may be at high-risk to develop lung cancer; limited data have been made available on such association, mostly from Japanese cohorts.

    Methods
    This retrospective multicentre study was conducted by the Groupe d’Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM”O”P), a collaborative group of about 200 French physicians dedicated to the study of rare pulmonary diseases. Patients presenting with CPFE syndrome and lung cancer at the referring centers from 2003 to 2012, were included. The clinical, pathological, and therapeutic features, as well as the outcome of patients, were collected and analyzed.

    Results
    A total of 47 patients presenting with lung cancer and CPFE syndrome were identified. All patients but one were men, with a mean age of 68 years. All patients were smokers, with a mean of 47 pack-years. The CPFE syndrome was diagnosed synchronously with lung cancer in 27 (57%) patients. Detection of lung cancer was incidental in 22 (47%) patients. The tumour was diagnosed at an early-stage, i.e. stage I-II, in 55% of cases. A pathological diagnosis of lung cancer was obtained for only 38 (81%) patients. Histological type was squamous cell carcinoma in 17 (36%) patients, adenocarcinoma in 14 (30%) patients, non-small cell lung cancer not otherwise specified in 3 (6%) patients, small cell lung cancer in 3 (6%) patients, and sarcomatoid carcinoma in one (2%) patient. There was no significant relation between tumor histology and location in the lung parenchyma (p=0.32). Overall, 20 of the 47 patients could not receive standard-of-care treatment for lung cancer, as per international recommendations and guidelines; this limitation was considered to be directly related to the CPFE syndrome in 8 (40%) cases. After a mean follow-up of 17 months, 35 patients were dead, and 12 patients were alive. Causes of death included locoregional or systemic tumor progression in 5 (14%) and 17 (49%) patients, respectively, respiratory failure in 8 (23%) patients, treatment-induced toxicity in 4 (11%) patients, and post-operative exacerbation of pulmonary fibrosis in 1 (3%) patient.

    Conclusion
    Lung cancer in patients with CPFE syndrome represents a specific entity characterized by very strong association with tobacco-smoking and male gender, peculiar histological-radiological presentation, poor prognosis due to major limitations and risks to conduct standard-of-care diagnostic and therapeutic interventions in a significant proportion of patients, and possible interest of screening. Lung cancer in CPFE syndrome further represents the most characteristic and severe model of tobacco-related disease.