Intralobar pulmonary sequestration with congenital cystic adematous malformation and rhabdomyomatous dysplasia

Pulmonary sequestration and congenital cystic adenomatous malformations (CCAM) are well known but still uncommon anomalies of the lung. Extralobar sequestrations are well described in association with CCAM, but fewer intralobar lesions are found with this association. The existence of striated muscle within CCAM is described, and we have evidence of dysplastic changes within the various cellular components occurring, with rhabdomyomatous dysplasia being one of these. A literature review shows no previous evidence of a reported intralobar sequestration associated with CCAM and rhabdomyomatous dysplastic changes.