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Review Article Open Access

Uncommon Diffuse Cystic Lung Diseases: Histopathological Insights

  • 1Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA
  • 2Division of Pathology, National Jewish Health, Denver, CO 80206, USA
+ Affiliations - Affiliations

Corresponding Author

Jian Jing, jian.jing@cuanschutz.edu

Received Date: May 05, 2026

Accepted Date: June 05, 2026

Abstract

Diffuse cystic lung diseases are infrequently encountered in pathology practice, yet when present, often pose significant diagnostic challenges. They typically follow unclassical or atypical radiological findings, requiring pathology for a definite diagnosis and appropriate management. Another common scenario involves specimens obtained following surgery for spontaneous pneumothorax, which are submitted as part of routine postoperative evaluation. The accurate diagnosis of cystic lung disease is critical, as it directly impacts patient management. While radiologic imaging often serves as the initial guide in identifying cystic lung lesions, histopathologic correlation is essential for definitive diagnosis. This article is centered on histology and aims to understand the histologic hallmarks and identification of some of these cystic lung entities, with the goal of helping pathologists to arrive at a confident and clinically meaningful diagnosis.

Keywords

Cystic lung disease, Histopathology, Intraparenchymal cysts, Lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, Pulmonary Langerhans cell histiocytosis, Lymphocytic interstitial pneumonia, Placental transmogrification, Differential diagnosis

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