Early pleuroparenchymal fibroelastosis mimicking lung malignancy: A case report

Our plain-English summary of Early pleuroparenchymal fibroelastosis mimicking lung malignancy: A case report, by H.S. Jung, H.J. Kim, K.W. Kim (World Journal of Clinical Cases, 2025).

The study

• Authors: H.S. Jung, H.J. Kim, K.W. Kim
• Journal: World Journal of Clinical Cases
• Published: 2025;13(32):111879
• Type of article: Case report
• Read it: PubMed · Publisher · Free full text

Abstract

Background: Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease affecting the upper lobes. Its atypical radiological appearance frequently mimics lung malignancy, complicating early diagnosis. This case highlighted the importance of histopathological confirmation to differentiate PPFE from malignant lesions.

Case Summary: A 62-year-old male with a significant smoking history presented with progressive dyspnea and a chronic nonproductive cough. High-resolution computed tomography revealed a localized fibrotic lesion in the left upper lobe with apical pleural thickening and subpleural consolidation. 18F-fluorodeoxyglucose positron emission tomography/computed tomography revealed moderate hypermetabolism (maximum standardized uptake value of 3.2), potentially indicating malignancy. Pulmonary function testing was deferred due to concurrent pneumothorax. The patient underwent video-assisted thoracoscopic surgery with segmental lung resection and talc pleurodesis. Histopathology confirmed dense fibroelastosis with abundant elastin deposition, minimal inflammation, and no evidence of malignancy. Differential diagnoses, including apical cap, chronic hypersensitivity pneumonitis, granulomatous infections, and asbestos-related disease were systematically excluded. Therefore, he was diagnosed with PPFE. Antifibrotic therapy with nintedanib was initiated postoperatively. At the 26-month follow-up, imaging and pulmonary function testing demonstrated stable disease with no recurrence of pneumothorax or functional decline.

Conclusion: Histopathology is essential for distinguishing PPFE from malignancy. Early diagnosis allows individualized therapy to slow progression.

©The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.

In plain English

This case report from South Korea described one patient: a 62-year-old Asian man, a former office worker who had smoked 50 pack-years and had no known occupational exposure to asbestos or silica. He had worsening shortness of breath for 1 week and a dry cough for 1 year. A chest X-ray showed a pneumothorax (air around the left lung). CT showed a lesion at the top of the left lung, and a PET scan showed moderate uptake (maximum standardized uptake value 3.2), which suggested cancer. Surgeons removed part of the lung and performed talc pleurodesis. Tissue examination showed dense fibroelastosis with abundant elastic fibers, little inflammation and no cancer cells. Tests for cancer-driving mutations were negative. The authors excluded other diagnoses, including asbestos-related fibrosis, citing the lack of relevant exposure and the tissue findings. The final diagnosis was idiopathic pleuroparenchymal fibroelastosis (PPFE), and the patient was started on nintedanib. At 26 months the patient was stable, with no return of the pneumothorax. The authors concluded that tissue confirmation is needed to tell PPFE apart from cancer. They noted that the missing baseline lung-function tests and the single case limit what can be concluded.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (single patient)
• Who was studied: One 62-year-old Asian man with a 50 pack-year smoking history, treated in the Department of Thoracic and Cardiovascular Surgery, CHA Bundang Medical Center, South Korea
• Exposure: No known occupational exposure to asbestos or silica; the patient was a former office worker. Asbestos was not the exposure under study.
• Main results:
  • Chest radiography showed a left pneumothorax
  • The PET/CT scan showed moderate fluorodeoxyglucose uptake (maximum standardized uptake value = 3.2) in the left upper lobe, suggesting malignancy
  • After video-assisted thoracoscopic surgery, tissue examination showed dense fibroelastosis with prominent elastic fibers, scant inflammation and no malignant cells
  • Molecular testing of the resected tissue was negative for driver mutations
  • Other diagnoses, including asbestos-related fibrosis, were ruled out on the basis of absent relevant exposure and the characteristic tissue findings
  • Final diagnosis was idiopathic PPFE of the left upper lobe
  • Nintedanib 150 mg orally twice daily was started after surgery
  • At 26 months after surgery the patient was clinically stable with no recurrence of pneumothorax
  • Lung function at 26 months: forced vital capacity 2.85 L and diffusing capacity (DLCO) 78% of predicted
  • The patient's Krebs von den Lungen-6 concentration was 230 U/mL, below the poor-prognosis threshold the authors cite
• The authors' conclusion: The authors concluded that tissue (histopathological) confirmation is needed to diagnose and manage PPFE that mimics lung cancer. They also stated that the positive outcome after antifibrotic therapy points to its potential use in early-stage PPFE.
• Limitations the authors state:
  • No baseline lung-function testing was done because of the initial pneumothorax, which limited assessment of early impairment and of the response to nintedanib
  • Findings from a single case cannot be generalized
  • Controlled trials are needed to confirm whether nintedanib works for this condition

Topics

Medical terms in this study, explained in our Lexicon: Interstitial Lung Disease, Dyspnea, Pleural Thickening, Consolidation, Pneumothorax, Video-Assisted Thoracoscopic Surgery (VATS), Talc Pleurodesis, Histopathology.

Related studies

• Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity? — C.D. Becker, J. Gil, et al. (2008)
• Pleuroparenchymal Fibroelastosis With Long History of Asbestos and Silicon Exposure — L. Xu, N. Rassaei, et al. (2018)
• A CARE-compliant case report: Lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis — H. Huang, R. Feng, et al. (2017)
• Asbestos exposure and tuberculous pleurisy as developmental causes of progressive unilateral upper-lung field pulmonary fibrosis radiologically consistent with pleuroparenchymal fibroelastosis — A. Sekine, E. Hagiwara, et al. (2021)
• Pleuroparenchymal sarcoidosis: A rare manifestation mimicking metastatic lung cancer — E. Gan, C.K. Tay (2024)
• Mesothelioma of the tunica vaginalis testis: a rare malignancy mimicking more common inguino-scrotal masses — P.J. Schure, K.C. van Dalen, et al. (2006)

Browse more research in World Journal of Clinical Cases, or return to medical research.