Cystic Lung Diseases Causing Spontaneous Pneumothorax

Abstract

Surgical specimens resulting from a spontaneous pneumothorax (SP) are commonly encountered in the general surgical pathology practice. Many of the SP cases are primary pneumothorax with no underlying lung diseases. Specimens with primary pneumothorax show nonspecific pneumothorax-related changes, which are important to recognize to exclude the true underlying lung diseases. A variety of disease entities may lead to diffuse cystic changes in the lungs, causing secondary pneumothorax. Some of the diseases are progressive and can cause irreversible damage to the lungs if not treated timely. Diagnosis of cystic lung diseases causing secondary pneumothorax is important for the treatment of the diseases and the prevention of future episodes of pneumothorax. Lymphangioleiomyomatosis and Langerhans cell histiocytosis are two common conditions causing diffuse cystic changes in the lungs. They are discussed in greater detail in this review, given their overlapping features in patient characteristics, radiological findings, and pathologic findings.