Question: I have a patient with biopsy-proven IPF who has been monitored with a yearly CT scan and has developed a 2 cm solitary pulmonary nodule. It does not appear spiculated. How aggressive should I be in treating this patient?

Answer: Lung cancer in IPF presents a proverbial "rock and a hard place" quandary, and is not uncommon. Le Jeune found that the rate of lung cancer in IPF patients is almost 5 times higher than in the population at large.1

 

Two things should be considered in evaluating how hard the "rocks" are. The first is that somewhere between 60 and 80% of patients with IPF will die of IPF but that also means that 20-40% will die of something else. A significant cause of death in this population is lung cancer, for which we have recognized therapies. A resection has the potential of being curative. But the combination of IPF and lung cancer poses heightened risk. Aubry et al reported that the mean survival of patients with IPF was 2.3 years from diagnosis but only 1.6 years for patients with IPF and lung cancer.2

The second key is, of course, the severity of the underlying interstitial disease and its influence on surgical outcome. Patients with IPF have high postoperative pulmonary morbidity and mortality, with ARDS being the primary concern. IPF patients with ARDS have a lower preoperative %FVC than those without postoperative ARDS.3 While the morbidity and mortality of patients with IPF is high, the surgical success in treating lung cancer in patients with IPF is similar to the success rate in patients without IPF, as reflected by similar rates of disease-free survival.4

Therefore the approach in a patient with a solitary pulmonary nodule and IPF is really no different than with patients with a nodule and in other lung diseases such as emphysema. The pros and cons of surgery have to be carefully discussed with the patient. If the patient is a reasonable candidate for a resection, the work up should be done as expeditiously as possible, as it would for any solitary pulmonary nodule.

  1. Le Jeune I, Gribbin J, West J, Smith C, Cullinan P, Hubbard R. The incidence of cancer in patients with idiopathic pulmonary fibrosis and sarcoidosis in the UK. Respir Med. 2007;101:2534-2540.
  2. Aubry MC, Myers JL, Douglas WW, et al. Primary pulmonary carcinoma in patients with idiopathic pulmonary fibrosis. Mayo Clin Proc. 2002;77:763-770.
  3. Kushibe K, Kawaguchi T, Takahama M, Kimura M, Tojo T, Taniguchi S. Operative indications for lung cancer with idiopathic pulmonary fibrosis. Thorac Cardiovasc Surg. 2007;55:505-508.
  4. Kawasaki H, Nagai K, Yoshida J, Nishimura M, Nishiwaki Y. Postoperative morbidity, mortality, and survival in lung cancer associated with idiopathic pulmonary fibrosis. J Surg Oncol. 2002;81:33-37.