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Year : 2014  |  Volume : 2  |  Issue : 1  |  Page : 33-36

A case of lung adenocarcinoma with idiopathic pulmonary fibrosis

1 Department of Pulmonary Medicine, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
2 Department of Radiotherapy, Burdwan Medical College and Hospital, Burdwan, West Bengal, India

Date of Web Publication5-Feb-2014

Correspondence Address:
Arnab Saha
D-60, Aurobinda Park, Bansdroni, Kolkata - 700 070, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-8775.126509

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Idiopathic pulmonary fibrosis (IPF) is a diffuse interstitial lung disease of unknown etiology. The prevalence of lung cancer among patients with IPF may vary between 4% and 9%. In India, there are is reported cases of IPF associated with lung cancer. In this case report, a 65-year-old male presented with gradually progressive dyspnea and dry cough and his high-resolution computed tomography scan of thorax revealed usual interstitial pneumonia with left lower lobe lung mass. Computed tomography-guided fine needle aspiration cytology of the mass was suggestive of adenocarcinoma. Patient was treated with chemotherapy.

Keywords: Adenocarcinoma, idiopathic pulmonary fibrosis, lung, lung adenocarcinoma

How to cite this article:
Saha A, Ghosh S, Saha K, Banerjee S, Mallick S. A case of lung adenocarcinoma with idiopathic pulmonary fibrosis. J Assoc Chest Physicians 2014;2:33-6

How to cite this URL:
Saha A, Ghosh S, Saha K, Banerjee S, Mallick S. A case of lung adenocarcinoma with idiopathic pulmonary fibrosis. J Assoc Chest Physicians [serial online] 2014 [cited 2022 Aug 7];2:33-6. Available from: https://www.jacpjournal.org/text.asp?2014/2/1/33/126509

  Introduction Top

Idiopathic pulmonary fibrosis (IPF) is a fibrotic inflammatory disorder of both lungs presenting with progressive dyspnea. [1] The disease is well-known for its relentless progression of fibrosis and early mortality. [1] However in some patients the clinical course is stable for prolonged periods even without treatment. [2] Majority of the patients suffer from severe dyspnea usually grade IV modified medical research council (MMRC) at diagnosis and the mean overall survival rate is 30%-50%. [3] Acute worsening of symptoms may be due to secondary infection, pulmonary embolism, pneumothorax, or heart failure. Sometimes accelerated phase may overlap on it. These patients also have an increased prevalence of lung cancer. [4],[5] Death occurs by hypoxia, respiratory failure, and ultimately cardiac failure.

  Case Report Top

A 65-year-old male smoker had attended to pulmonary medicine outdoor with shortness of breath for last 6 months which was insidious in onset, gradually progressive and on admission severity was MMRC grade IV. Paroxysmal nocturnal dyspnea, wheeze, and post nasal drip was absent. A gradually progressive dry cough for last 4 months with increase in intensity for last 1 month was also present. Hemoptysis, fever, and chest pain were absent. He gave history of loss of appetite and about 10 kg weight loss in last 3 months. He denied any history suggestive of Raynaud's phenomenon, joint pain, skin rashes, oral ulcers, skin tightening, and dryness of mouth.

On general survey, there was presence of pallor, third degree clubbing. There was neither engorgement of neck veins nor any lymphadenopathy. On respiratory system examination, impaired percussion note, diminished vocal resonance, and diminished vesicular breath sound elicited over both interscapular areas which was associated with velcro-like fine end inspiratory crackles over bilateral infraaxillary and infrascapular areas. Other system examination was normal.

On investigation, the complete blood count was normal except normocytic normochromic anemia with hemoglobin 9.5 g/dL. His blood sugar, renal, and liver function were normal. The connective tissue disease (CTD) profile [anti cyclic citrullinated peptide antibodies (anti-CCP), Antinuclear antibody (ANA), anti-ds-DNA, anti-sm- antibody, anti Scl-70, anti-centromere antibody, anti-Ro (SS-A), anti-La (SS-B), anti U 1 -RNP] was negative. Two-dimentional echocardiography showed no abnormality. The chest x-ray showed bilateral lower zone reticular opacity with presence of a homogenous opacity involving left mid zone of lung field [Figure 1]. High-resolution computed tomography thorax revealed a mass lesion in left lower lobe possibly a bronchogenic growth with the presence of septal thickening and basal predominant subpleural reticulation suggestive of usual interstial pneumonia pattern (UIP) [Figure 2]. Pulmonary function showed restrictive pattern. As his all CTD profile was negative the patient was diagnosed as IPF. Then we did computed tomography (CT)-guided fine needle aspiration cytology (FNAC) from the left lower lobe mass lesion. The FNAC was of suggestive of adenocarcinoma [Figure 3]. The patient was already on treatment with azathioprine and prednisolone for IPF for nearly 6 months. He was started with chemotherapy with paclitaxel and carboplatin for lung cancer. He tolerated the first cycle of chemotherapy well. But he did not come further for next cycle. As per his relative's report he had developed respiratory distress and severe weakness and he was unwilling to take repeat chemotherapy.
Figure 1: Chest X-ray showing bilateral reticular opacities involving all the zones of lung with a mass at left lower zone close to hilum

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Figure 2: (a) Lung window of high-resolution computed tomography thorax showing bilateral basal predominant reticulations, interlobular septal thickening with honeycombing suggestive of usual interstial pneumonia pattern and (b) Mediastinal window showing a mass lesion in left lower lobe possibly a bronchogenic growth

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Figure 3: Fine needle aspiration cytology of the lung mass showing columnar, cuboidal, or polygonal cells with round to oval nucleus and varying quantities of fine vacuolated cytoplasm; often eccentric with high nuclear/cytoplasm ratio, and finely granular chromatin suggestive of adenocarcinoma

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  Discussion Top

IPF was defined as: (1) Chronic fibrosing interstitial pneumonia without known etiology such as drug toxicity, environmental exposures, or collagen vascular diseases; (2) impaired pulmonary function (restrictive defect and impaired gas exchange); (3) HRCT showing typical patterns of UIP such as subpleural reticular opacities or honeycombing lesions predominantly in lower lobes; and (4) pathological confirmation by surgical (open or thoracoscopic) lung biopsy suggestive of usual interstitial pneumonia. [1],[6]

The common mode of presentation of IPF is dyspnea and dry cough. Diagnosis of IPF is usually based on symptoms, classical findings like finger and toe clubbing and bibasilar velcro crepitation on auscultation of chest. Chest X ray and HRCT scan add to the diagnosis. The common and classical radiographic abnormality in IPF is peripheral reticular opacities most marked at the bases, often associated with honeycombing and lower lobe volume loss. [1] HRCT scan of thorax describes the lesions as reticular opacities (intralobular opacities), honeycombing, traction bronchiectasis, and bronchiolectasis and ultimately architectural distortion of lung parenchyma. [1]

It was found to have an increased risk of lung cancer in IPF cases in comparison to general population. Hubbard et al., [7] reported increased risk of lung cancer in IPF patients compared to age- and sex-matched control population in a large population-based cohort study. The findings show that the relative risk of lung cancer was increased sevenfold in the IPF group, compared with the controls, and the excess risk changed little after adjustment for cigarette smoking. Comparing nonsmokers and current smokers, the relative risk of lung cancer associated with IPF was twice as high in the nonsmokers. In an earlier study of patients at the Brompton Hospital in London, Turner-Warwick et al., [8] found lung cancer in 20 of 205 IPF patients and estimated a 14-fold increased risk of lung cancer in IPF patients in comparison with the general population.

The prevalence of lung cancer among patients with IPF may vary between 4% and 9%. [7],[8],[9] In a retrospective cohort study, the cumulative incidence of lung cancer among patients with IPF was 3.3% at 1 year, 15.4% at 5 years, and 54.7% at 10 years. [10]

Assuming that IPF is associated with an increased risk of lung cancer, the mechanism is not certain. There are three hypotheses to explain the relationship: (1) pulmonary fibrosis causes lung cancer, (2) lung cancer and/or its treatment causes pulmonary fibrosis, and/or (3) common mediators cause pulmonary fibrosis and a lung cancer. [11] Because cigarette smoking itself is also an independent risk factor for both IPF and lung cancer, smoking can be a cause for the association of both diseases. The risk of developing lung cancer is greater for patients with IPF who are male ever smokers. [7],[9],[10] Second, there may also be other confounding factors for both conditions, like known (asbestos or silica) or unknown occupational (metal dust) exposure. Epidemiologic studies involving follow-up of asbestos workers and persons with IPF have generally confirmed this hypothesis. [7],[12],[13] CTDs involving lung tissue also have increased risk of lung cancer. [14]

Another possibility is that fibrosis itself may contribute to the development of cancer. The diffuse inflammatory process of IPF may be involved in the carcinogenesis by the occurrence of atypical or dysplastic epithelial changes in pulmonary fibrosis which progressed to invasive malignancy. [15] In that situation, the cancer will develop in the region not far from the area of major fibrosis.

Another promising but not new concept is that new vessel formation or angiogenesis with generation and proliferation of immortal myofibroblast is associated with transformation of cancer cells. [16] It was first noticed by Turner and Warwick [17] in 1963 where they described presence of anastomoses between systemic and pulmonary microvsculature in lungs of patients with IPF. This angiogenesis is presumed to be an imbalance between angiogenic [Members of CXC chemokine family (CXCL8, CXCL5), vascular endothelial growth factor (VEGF), bFGF (basic fibroblast growth factor)], and angiostatic (CXCL10) factors. [18],[19],[20] Hypoxia is an initiating factor for VEGF expression.

There was no significant difference in the percentage of patients with other abnormal serum tumor markers, except that the percentage of patients with abnormal nonspecific enolase was significantly higher in the carcinoma group than in the IPF group (28.6% vs. 7.4%, P = 0.041). [21]

When lung cancer is associated with IPF, the additional symptoms are hemoptysis, chest pain, and sputum. When cancer is developed over the disease process of fibrosis, the main diagnostic feature in chest X-ray is a large opacity presenting as a mass lesion over the prevailing reticular opacities. As the area of affection is at bases and at periphery, lung cancer is commonly found at lower lung lobes. However in some another studies, the preferable area of affection is in the upper lobes and in the nonfibrotic area as per chest CT scan findings. [5] The mainstay of cancer diagnosis is by CT-guided FNAC. The common cytological type is adenocarcinoma [22] but in some another studies squamous cell carcinoma is also found as common etiology. [5]

Mortality is high in IPF patients. Muller and Silva mention that the overall survival rate is 30%-50%. [3] The median length of survival from time of diagnosis varies between 2.5 to 3.5 years. [1] The overall median survival of IPF-CA patients is 7 months (range 2.51-11.49) in comparison with IPF only patients (14 months, range 1.26-26.74). [21]

Chemotherapy sometimes causes acute exacerbation of IPF. [23]

  References Top

1.American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society. International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002;165:277-304.  Back to cited text no. 1
2.Lynch JP 3 rd , Saggar R, Weigt SS, Zisman DA, White ES. Usual interstitial pneumonia. Semin Respir Crit Care Med 2006;27:634-51.  Back to cited text no. 2
3.Muller NL, Silva CI. Idiopahic pulmonary fibrosis. In: Muller NL, Silva CI, editors. Imaging of the Chest. 1 st ed. Philadelphia: Saunders; 2008. p. 629-43.  Back to cited text no. 3
4.Aubry MC, Myers JL, Douglas WW, Tazelaar HD, Washington Stephens TL, Hartman TE, et al. Primary pulmonary carcinoma in patients with idiopathic pulmonary fibrosis. Mayo Clin Proc 2002;77:763-70.  Back to cited text no. 4
5.Park J, Kim DS, Shim TS, Lim CM, Koh Y, Lee SD, et al. Lung cancer in patients with idiopathic pulmonary fibrosis. Eur Respir J 2001;17:1216-9.  Back to cited text no. 5
6.Raghu G. Interstitial lung disease: A diagnostic approach. Are CT scan and lung biopsy indicated in every patient? Am J Respir Crit Care Med 1995;151:909-14.  Back to cited text no. 6
7.Hubbard R, Venn A, Lewis S, Britton J. Lung cancer and cryptogenic fibrosing alveolitis. A population-based cohort study. Am J Respir Crit Care Med 2000;161:5-8.  Back to cited text no. 7
8.Turner-Warwick M, Lebowitz M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis and lung cancer. Thorax 1980;35:496-9.  Back to cited text no. 8
9.Harris JM, Jhonston ID, Rudd R, Taylor AJ, Cullinan P. Cryptogenic fibrosing alveolitis and lung cancer: The BTS study. Thorax 2010;65:70-6.  Back to cited text no. 9
10.Ozawa Y, Suda T, Natio T, Enomoto N, Hashimoto D, Fujisawa T, et al. Cumulative incidence and predictive factors for lung cancer in IPF. Respirology 2009;14:723-8.  Back to cited text no. 10
11.Daniels CE, Jett JR. Does interstitial lung disease predispose to lung cancer? Curr Opin Pulm Med 2005;11:431-7.  Back to cited text no. 11
12.Harris JM, Cullinan P, McDonald JC. Does cryptogenic fibrosing alveolitis carry an increased risk of death from lung cancer? J Epidemiol Community Health 1998;52:602-3.  Back to cited text no. 12
13.Baumgartner KB, Samet JM, Stidley CA, Colby TV, Waldron JA. Cigarette smoking: A risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1997;155:242-8.  Back to cited text no. 13
14.Yang Y, Fujita J, Tokuda M, Bandoh S, Ishida T. Lung cancer associated with several connective tissue diseases: With a review of literature. Rheumatol Int 2001;21:106-11.  Back to cited text no. 14
15.Meyer EC, Liebow AA. Relationship of interstitial pneumonia honeycombing and atypical epithelial proliferation to lung cancer. Cancer 1965;18:322-51.  Back to cited text no. 15
16.Tzouvelekis A, Anevlavis S, Bouros D. Angiogenesis in interstitial lung diseases: A pathogenetic hallmark or a bystander? Respir Res 2006;7:82.  Back to cited text no. 16
17.Turner-Warwick M. Precapillary systemic-pulmonary anastomoses. Thorax 1963;18:225-37.  Back to cited text no. 17
18.Keane MP, Arenberg DA, Lynch JP 3 rd , Whyte RI, Iannettoni MD, Burdick MD, et al. The CXC chemokines, IL-8 and IP-10, regulate angiogenic activity in idiopathic pulmonary fibrosis. J Immunol 1997;159:1437-43.  Back to cited text no. 18
19.Koch AE, Harlow LA, Haines GK, Amento EP, Unemori EN, Wong WL, et al. Vascular endothelial growth factor. A cytokine modulating endothelial function in rheumatoid arthritis. J Immunol 1994;152:4149-56.  Back to cited text no. 19
20.Meyer KC, Cardoni A, Xiang ZZ. Vascular endothelial growth factor in bronchoalveolar lavage from normal subjects and patients with diffuse parenchymal lung disease. J Lab Clin Med 2000;135:332-8.  Back to cited text no. 20
21.Xu Y, Zhong W, Zhang L, Zhao J, Li L, Wang M. Clinical characteristics of patients with lung cancer and idiopathic pulmonary fibrosis in China. Thoracic Cancer 2012;3:156-61.  Back to cited text no. 21
22.Samet JM. Does idiopathic pulmonary fibrosis increase lung cancer risk? Am J Respir Crit Care Med 2000;161:1-2.  Back to cited text no. 22
23.Isobe K, Hata Y, Sakamoto S, Takai Y, Shibuya K, Homma S. Clinical characteristics of acute respiratory deterioration in pulmonary fibrosis associated with lung cancer following anti cancer therapy. Respirology 2010;15:88-92.  Back to cited text no. 23


  [Figure 1], [Figure 2], [Figure 3]


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