title: Diffuse chondroid malformation of the lung: cases series of a hitherto undescribed congenital lung disease.
abstract: Congenital chondroid lesions of the lung are rare pathological findings. They are a constant feature of lung malformations such as giant cystic pulmonary chondroid hamartoma, chondroid cystic malformation, and in the "cartilaginous variant" of congenital adenomatoid malformation. All of these present as a large single thoracic mass.We present the cases of three males and two females with hitherto undescribed diffuse chondroid lung disease, all but one of whom had neonatal respiratory distress syndrome with interstitial syndrome on chest radiograph. The pathological findings were similar in all patients, showing large areas of disorganized lung parenchyma containing diffusely distributed mature cartilage islands. With a mean follow-up of 6 years, all patients had a favorable outcome. This diffuse chondroid lung disease appears to be a new entity whose initial presentation mimicked interstitial lung disease without the usual clinical, radiological, and histological features. We speculate that it could be part of a clinical spectrum between malformative chondroid lung cyst and congenital pulmonary airway malformation.
authors.affiliation: Department of Pediatric Pulmonology, Hôpital Femme Mère Enfant, Bron, France. philippe.reix@chu-lyon.fr
authors.forename: Philippe
authors.initials: P
authors.lastname: Reix
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Wanin
authors.forename: Fréderique
authors.initials: F
authors.lastname: Dijoud
authors.forename: Jacques
authors.initials: J
authors.lastname: Brouard
authors.forename: Ralph
authors.initials: R
authors.lastname: Epaud
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Pracros
authors.forename: Liliane
authors.initials: L
authors.lastname: Boccon-Gibod
authors.forename: Gabriel
authors.initials: G
authors.lastname: Bellon
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D008171:Lung Diseases / Q000151:congenital* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D011859:Radiography; D012127:Respiratory Distress Syndrome, Newborn / Q000000981:diagnostic imaging / Q000473:pathology; D016896:Treatment Outcome