Early pleuroparenchymal fibroelastosis mimicking lung malignancy: A case report

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.

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.

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