Elsevier

Mayo Clinic Proceedings

Volume 77, Issue 8, August 2002, Pages 763-770
Mayo Clinic Proceedings

Original Article
Primary Pulmonary Carcinoma in Patients With Idiopathic Pulmonary Fibrosis

https://doi.org/10.4065/77.8.763Get rights and content

Objective

To identify distinguishing characteristics between patients with idiopathic pulmonary fibrosis (IPF) and primary lung carcinoma and patients with either IPF or carcinoma alone.

Patients and Methods

The study group consisted of 24 patients with histologically proven usual interstitial pneumonia and lung carcinoma identified through a search of the Rochester Mayo Clinic database for 1990 to 1998. Medical records, radiographs, and histological slides were reviewed. Several variables including survival were compared in 2 control groups, IPF only and carcinoma only, by using various statistical methods.

Results

Our study group included 21 men and 3 women (mean age, 72.3 years). Twenty-two were past or current smokers. Approximately half of the lung carcinomas were incidental findings. Of the 14 patients with preoperative computed tomographic scans, 12 had peripheral tumors situated in areas of fibrosis. Squamous cell carcinoma was the most common histological type, accounting for 16 cases. Almost all patients underwent surgical treatment; nearly 40% developed postoperative complications, and 3 died within 30 days of surgery. The ratio of men to women in patients with IPF and carcinoma was 7:1 compared with 1:1 in patients with IPF only (P=.003). Patients with IPF and carcinoma were also older, with a mean age of 72.3 years compared with 64.4 years (P=.001), and were more often smokers (P=.002). Carcinomas involved the lower lobes in 42% of patients with IPF and carcinoma compared with 29% of patients with carcinoma only (P=.004) and were mainly composed of squamous cell carcinoma (P=.004). Mean survival in patients with IPF and lung carcinoma was 2.3 years after the diagnosis of IPF and 1.6 years after that of carcinoma. This finding did not differ significantly from survival of patients with either IPF or carcinoma alone. However, statistical power was limited.

Conclusion

Carcinoma in patients with IPF arises in older male smokers and usually presents as peripheral squamous cell carcinoma. The prognosis is poor.

Section snippets

PATIENTS AND METHODS

Our study group consisted of 24 patients with both histologically proven UIP and primary lung carcinoma. Patients were identified through a retrospective computerized search of the Rochester Mayo Clinic database for 1990 to 1998. Patients with connective tissue disease, asbestos exposure, previous radiation therapy, or metastatic disease to the lung were excluded. Patients taking drugs that could potentially induce pulmonary fibrosis were also excluded. Thus, study patients were considered to

Clinical Findings

Clinical findings in the 24 patients with IPF and carcinoma are detailed in Table 1. The study group was composed of 21 men and 3 women, with a mean age of 72.3 years. Twenty were past and 2 were current cigarette smokers, with mean exposures of 53.5 pack-years (range, 10-105 pack-years). Sixteen patients (67%) had IPF and carcinoma diagnosed within 4 months of one another. In 6 of these, both diagnoses were made during the same evaluation. In 7, the diagnosis of carcinoma followed the

DISCUSSION

Carcinoma arising in association with IPF appears to be more common in men than women. The male predominance in our study parallels that seen in other reports of lung carcinoma developing in patients with IPF or other types of chronic interstitial fibrosis. In a review of 4 published series1, 11, 12, 21 describing a total of nearly 150 patients, men outnumbered women by a ratio of more than 10:1. This greatly exceeds the male-female ratio generally observed in IPF22 or primary lung carcinoma

Acknowledgments

We thank Jennifer A. Besse for her assistance with statistics in this study.

REFERENCES (30)

  • M Turner-Warwick et al.

    Cryptogenic fibrosing alveolitis and lung cancer

    Thorax

    (1980)
  • F Beaumont et al.

    Simultaneous occurrence of pulmonary interstitial fibrosis and alveolar cell carcinoma in one family

    Thorax

    (1981)
  • PS Shankar

    Cryptogenic fibrosing alveolitis and carcinoma of the lung

    Indian J Med Sci

    (1983)
  • H Kuhara et al.

    Synchronous bilateral double primary lung cancer associated with diffuse interstitial fibrosing pneumonitis (DIFP)

    Acta Pathol Jpn

    (1984)
  • T Kawai et al.

    Diffuse interstitial pulmonary fibrosis and lung cancer

    Acta Pathol Jpn

    (1987)
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