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Pathologica. 2016 Mar;108(1):38-41.

Type II congenital pulmonary airway malformation associated with intralobar pulmonary sequestration: report of a case and review of classification criteria.

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Department of Medical Biotechnologies, Pathology Unit, University of Siena, Siena, Italy.
Department of Medical and Surgical Sciences and Neurosciences, University of Siena, Siena, Italy.
Pathology Unit, "Azienda Ospedaliera Universitaria Senese", Siena, Italy.


Pulmonary congenital abnormalities are rare disorders including congenital pulmonary airway malformations (CPAM) and pulmonary sequestration (PS). CPAM is a lesion characterized by the presence of anomalous bronchiolar or acinar structures, variable in size, either cystic or not cystic. PS is generally defined as nonfunctioning lung tissue that is not in normal continuity with the tracheobronchial tree and that derives its blood supply from systemic vessels. We describe a case of a baby girl with a very rare association between CPAM type 2 and intralobar pulmonary sequestration (IPS) focusing on the cystic lesions typical of CPAM and on the lymphatic and blood vessels. The cells lining the cysts often were positive for D2-40 (oncofetal protein M2A). Lymphatic endothelial cells, positive for D2-40, were widely present in the lung parenchyma and dilated lymphatic vessels were present also in the inter-alveolar septa. Moreover, we discuss the pathogenesis of CPAM and its classification criteria.


Congenital cystic adenomatoid malformation; Congenital pulmonary airway malformation; Congenital thoracic malformations; Endoderm/mesoderm interaction; Lung cysts; Pulmonary sequestration

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