Jung HS, Kim HJ, Kim KW. Early pleuroparenchymal fibroelastosis mimicking lung malignancy: A case report. World J Clin Cases 2025; 13(32): 111879 [DOI: 10.12998/wjcc.v13.i32.111879]
Corresponding Author of This Article
Kwan Wook Kim, MD, PhD, Department of Thoracic and Cardiovascular Surgery, CHA Bundang Medical Center, No. 59 Yatap-ro, Bundang-gu, Seongnam-si 13496, South Korea. mujin100km@cha.ac.kr
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Respiratory System
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Case Report
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This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Nov 16, 2025 (publication date) through Nov 15, 2025
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World Journal of Clinical Cases
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Jung HS, Kim HJ, Kim KW. Early pleuroparenchymal fibroelastosis mimicking lung malignancy: A case report. World J Clin Cases 2025; 13(32): 111879 [DOI: 10.12998/wjcc.v13.i32.111879]
World J Clin Cases. Nov 16, 2025; 13(32): 111879 Published online Nov 16, 2025. doi: 10.12998/wjcc.v13.i32.111879
Early pleuroparenchymal fibroelastosis mimicking lung malignancy: A case report
Hee Suk Jung, Hyun Jung Kim, Kwan Wook Kim
Hee Suk Jung, Hyun Jung Kim, Kwan Wook Kim, Department of Thoracic and Cardiovascular Surgery, CHA Bundang Medical Center, Seongnam-si 13496, South Korea
Author contributions: Jung HS and Kim KW contributed to the manuscript writing and editing and data collection; Kim HJ contributed to the data analysis; Kim KW contributed to the conceptualization and supervision of the report; all authors read and approved the final manuscript.
Informed consent statement: Written informed consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Kwan Wook Kim, MD, PhD, Department of Thoracic and Cardiovascular Surgery, CHA Bundang Medical Center, No. 59 Yatap-ro, Bundang-gu, Seongnam-si 13496, South Korea. mujin100km@cha.ac.kr
Received: July 14, 2025 Revised: August 18, 2025 Accepted: October 10, 2025 Published online: November 16, 2025 Processing time: 124 Days and 4.2 Hours
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.
Core Tip: Pleuroparenchymal fibroelastosis (PPFE) frequently mimics lung malignancy on radiographs, leading to challenges in diagnosing PPFE early. This case illustrated that the localized fibrotic lesions with moderate fluorodeoxyglucose uptake on positron emission tomography/computed tomography initially suggested malignancy. However, after surgical biopsy and histopathological examination including elastic fiber staining, the patient was accurately diagnosed with PPFE. Early suspicion of PPFE and initiation of antifibrotic therapy, such as nintedanib, may effectively stabilize early-stage disease and prevent progression.
