Although the role of ER-stress in tumourigenesis is controversial [69,70], recent evidence showing that ER stress may attenuate senescence and promote tumorigenesis might explain the co-occurrence of LC (histological subtype ADC) and pulmonary fibrosis in families with an SFTPA1/2 mutation [71]. The gene discussed is SFTPA1; the disease is laryngotracheoesophageal cleft.