Clinical features and outcomes of patients with lung cancer as well as combined pulmonary fibrosis and emphysema

Abstract

Abstract. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) has been characterized by severely impaired gas exchange and poor survival. However, the clinical features of patients with lung cancer plus CPFE have remained elusive. The present study performed a retrospective analysis to examine the clinical characteristics and outcome of surgically resected patients with lung cancer plus CPFE. Among 831 patients with primary lung cancer who underwent surgical resection, 23 patients (2.8%) were diagnosed with CPFE and 9 patients (1.1%) with solely idiopathic pulmonary fibrosis (IPF). Thirty-five patients were stratified as the solely emphysema group with adjustment of the pathological stage. The clinicopathological characteristics of patients in the CPFE group and their outcomes were evaluated and compared with those with the solely IPF or solely emphysema groups. Within the CPFE group, no significant differences in survival between patients with post-operative acute exacerbation (AE; n=3) and those without AE (n=20) were noted; however, in the solely IPF group, patients with post-operative AE (n=4) had a significantly shorter survival than those without AE (n=5; P=0.022). The 5-year survival rate of patients in the CPFE, solely IPF and solely emphysema groups was 22, 22 and 58%, respectively. Furthermore, the CPFE and solely IPF groups showed a significantly shorter survival than the solely emphysema group (P=0.001 and 0.011, respectively). In conclusion, surgically resected lung cancer patients with CPFE had poor survival, which was, in contrast to that of lung cancer patients with solely IPF, not affected by AE. IntroductionThe syndrome of combined pulmonary fibrosis and emphysema (CPFE) has been suggested to be a clinically important phenotype of pulmonary fibrosis (1). Cottin et al (1) defined CPFE as a distinct condition, with characteristic features in the chest computed tomography (CT) image that include emphysema in the upper pulmonary region concomitant with parenchymal fibrosis in the lower region. The syndrome is characterized by sub-normal spirometry, severely impaired gas exchange, high prevalence of pulmonary hypertension and poor survival (2-4). Mejía et al (3) reported that survival of CPFE patients was poorer compared with that of patients with idiopathic pulmonary fibrosis (IPF) alone and that pulmonary hypertension in CPFE patients was an independent predictor of mortality. Previous studies have indicated that CPFE patients have a significantly increased risk of lung cancer compared with patients with chronic obstructive pulmonary disease (COPD) or IPF alone (1,4,5). Post-operative acute exacerbation (AE) of interstitial lung disease in patients with lung cancer is associated with a high mortality rate (43.9%) (6). However, the clinical features and prognosis of patients with lung cancer and CPFE have remained elusive. The present study performed a retrospective analysis to determine the prevalence, clinical characteristics and outcome of patients with surgically resected lung canc...

IPF and CPFE—The Two Different Entities or Two Different Presentations of the Same Disease?

“…Like previously reported, cancer had mainly subpleural localization, in the lower lobes, while in patients with emphysema -in the upper lobes [36][37][38]. Furthermore, a 5-year survival rate did not differ between the CPFE and IPF group (22% and 22%, respectively) [39]. Similar results were reported by Sato et al [40].…”

“…Interestingly, according to scarce data, squamous cell cancer followed by adenocarcinoma were the most common types in the CPFE group [39,40]. In the IPF group, that sequence may be reversed [39,40,42].…”

Clinical characteristics and outcomes of lung cancer patients with combined pulmonary fibrosis and emphysema: a systematic review and meta-analysis of 13 studies

“…Several previous studies showed lung cancer in patients with CPFE was advanced stage (10,14), while other studies indicated that more lung cancer patients with CPFE were early stage (11)(12)(13)(15)(16)(17)(18)(19)(20)(21)(22). Pulmonary function test was conducted in several studies, and the results have varied (11)(12)(14)(15)(16)(17)(18)(19)(20)(21)(22). CPFE was an independent factor for a poor prognosis in lung cancer patient with CPFE (11,18,21).…”

“…In three previous studies of lung cancer patients with CPFE adenocarcinoma was the most common type of cancer, followed by squamous cell carcinoma (10)(11)(12). However, other studies had shown that squamous cell carcinoma was the most common type of cancer (13)(14)(15)(16)(17)(18)(19)(20)(21)(22). Several previous studies showed lung cancer in patients with CPFE was advanced stage (10,14), while other studies indicated that more lung cancer patients with CPFE were early stage (11)(12)(13)(15)(16)(17)(18)(19)(20)(21)(22).…”

“…However, other studies had shown that squamous cell carcinoma was the most common type of cancer (13)(14)(15)(16)(17)(18)(19)(20)(21)(22). Several previous studies showed lung cancer in patients with CPFE was advanced stage (10,14), while other studies indicated that more lung cancer patients with CPFE were early stage (11)(12)(13)(15)(16)(17)(18)(19)(20)(21)(22). Pulmonary function test was conducted in several studies, and the results have varied (11)(12)(14)(15)(16)(17)(18)(19)(20)(21)(22).…”