J.Jpn. Surg. Soc.. 124(6): 485-491, 2023

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HISTOLOGICAL CLASSIFICATION OF CONGENITAL CYSTIC LUNG DISEASES : UPDATE

Congenital cystic lung diseases (CCLDs) have been detected and resected following pneumonia. Now, most CCLDs are diagnosed during the fetal period and the lesions are resected at an appropriate time, often before inflammatory events, based on highly accurate imaging studies, and detailed histological examination of well-preserved specimens is also possible. Recent advances in molecular analytics, including comprehensive methods, have revealed several causative genes of CCLDs. The histological classification of some types of CCLDs has been revised after reassessment of the histological findings and new molecular findings. Among the five types of congenital pulmonary airway malformation (CPAM), a hotspot variant in KRAS was detected in patients with type 1 CPAM and in some patients with type 3 CPAM, and alterations in DICER1 were detected in patients with type 4 CPAM. These types are now characterized by preneoplastic lesions. Microcystic parenchymal maldevelopment, which is characterized by histological changes associated with bronchial atresia during the fetal period, often occurs in type 2 CPAM and in patients with intrapulmonary or extrapulmonary sequestrations, and many patients with type 2 CPAM have since been reclassified as having bronchial atresia. Accurate histological diagnosis can help clinicians determine the appropriate therapeutic strategy.

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