Abstract. Idiopathic pulmonary fibrosis (IPF) is a rare interstitial lung disease limited to the lung with an undefined etiopathogenesis and a very short life expectancy (less than 5 years). IPF susceptibility has been associated with several genetic and environmental risk factors and the prognosis isIdiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with poor prognosis and an undefined etiopathogenesis. Several genetic and environmental risk factors for IPF have been identified including cigarette smoking, metal dust and other pollutant exposure, genetic mutations and polymorphisms (1-5). IPF prognosis is really poor (less than 5 years) and is conditioned by comorbidities negatively affecting patients' quality of life and survival (e.g. gastro-esophageal reflux, depression, venous thromboembolism, pulmonary hypertension and other cardiovascular disorders, and lung cancer) (6-10). The incidence of pulmonary neoplasm is much higher in IPF patients than in the general population (11). Ozawa et al. (12) for instance, reported 3.3%, 15.4% and 54.7% of lung cancer incidence in IPF patients after one, five and ten years of follow-up, respectively (12). Common mechanisms shared by the two diseases remain unknown. An aberrant expression of miRNAs regulating non-small cell lung cancer (NSCLC) and IPF has been suggested together with a crucial role of tyrosine kinase inhibitor directed against growth factors (plateletderived growth factor, vascular endothelial growth factor and fibroblast growth factor) (13,14).Lung cancer (LC) significantly reduces survival of IPF patients, including patients with combined pulmonary emphysema and pulmonary fibrosis (15). It generally occurs in the peripheral areas of the lower lobes, where fibrotic changes are predominant. In particular, LC develops from honeycomb areas or in the border between honeycombing and non fibrotic areas and the squamous cell carcinoma is the predominant histological type (16,17). Invasive diagnostic or therapeutic procedures for neoplasms promote the development of Acute exacerbations (AE) in 80% of IPF patients and the lung surgical treatment puts about 1/5 of them at high risk of death (17,18). A perioperative Pirfenidone treatment has recently been proposed to reduce the incidence of postoperative acute events (18)(19)(20). In this context, the therapeutic management of LC is a difficult task and is still questioned, especially in IPF patients with severe fibrotic involvement, whether the tumor should be treated or not (21,22). Although Lee et al. (15) reported that radiotherapy and surgical treatments of LC result in a reduced survival of IPF patients, according to Kumar et al. (19) lung resection in selected cases can improve their survival. Furthermore, Tomassetti and colleagues (21) have suggested that in mild IPF patients the sublobar resection in 773