Abstract
PURPOSE: Patients with combined pulmonary fibrosis and emphysema (CPFE) have been suggested to have an increased risk of lung cancer. We conducted a systematic review of all published data and performed a meta-analysis to define the characteristics of lung cancer that develops in CPFE. METHOD: We searched Pubmed, Embase, and Cochrane to find original articles about lung cancer and CPFE published prior to September 2015. All titles/abstracts were reviewed by two radiologists to identify articles that used predefined selection criteria. Summary estimates were generated using a random-effect model and odds ratios (ORs) to develop squamous cell carcinoma (SqCC) were calculated. Kaplan-Meier survival curves were obtained for the survival of patients with CPFE and non-CPFE. RESULTS: Nine original articles that assessed 620 patients were included in this review. In the pooled data, patients were older age (70.4 years), almost all were heavy smokers (53.5 pack years), and males were predominant (92.6%). SqCC was the most common type (42.3%), followed by adenocarcinoma (34.4%). Compared with lung cancer population with an otherwise normal lung, the OR to develop SqCC in CPFE was 9.06 (95% CI, 6.08-13.5). The ORs in CPFE compared with lung cancers that developed in lungs with fibrosis or emphysema were also higher. The median survival for CPFE patients with lung cancer (19.5 months) was significantly shorter than in non-CPFE (53.1 months). CONCLUSIONS: Lung cancer in CPFE, most commonly SqCC, presents in elderly heavy smokers with a male predominance. The median survival for CPFE patients with lung cancer is 19.5 months.