Cystic lung disease

Abstract

Abstract Cystic lung diseases are a group of rare disorders that can solely involve the lungs or might be part of a multi-organ process. Multidisciplinary assessment, including lung histology, is key to reaching the final diagnosis, as some might be hereditary and linked to a risk of malignancy. Tissue for microscopic examination of the lungs is mainly accessed during surgical treatment for pneumothorax, and can suggest an underlying cystic disease. This multidisciplinary approach is reflected in this review article, with summaries of the clinico-radiological presentation of cystic lung disease and detailed histopathological descriptions of these uncommon conditions, namely lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, Birt-Hogg-Dube syndrome, lymphocytic interstitial pneumonia, pulmonary amyloid and light chain deposition disease and metastases of sarcomas.