Congenital cystic adenomatoid malformation of the lung (CCAM): Evaluation of the cellular components

Congenital cystic adenomatoid malformation of the lung (CCAM) is a rare congenital lesion whose pathogenesis is not well defined. It is generally accepted that the various types of CCAMs originate at different levels of the tracheobronchial tree. To further define the pathogenesis of CCAM, we evalua...

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Bibliographic Details
Published in:Human pathology 1999-06, Vol.30 (6), p.618-625
Main Authors: Morotti, Raffaella A, Cangiarella, Joan, Gutierrez, Maria C, Jagirdar, Jaishree, Askin, Fred, Singh, Gurmukh, Profitt, Sherri A, Wert, Susan E, Whitsett, Jeffrey A, Greco, M.Alba
Format: Article
Language:English
Subjects:
Antigens, Neoplasm - metabolism
Biological and medical sciences
CD5 Antigens - metabolism
Clara cell antigen
congenital cystic adenomatoid malformation
Cystic Adenomatoid Malformation of Lung, Congenital - metabolism
Cystic Adenomatoid Malformation of Lung, Congenital - pathology
Fetal Diseases - metabolism
Fetal Diseases - pathology
Fetus - abnormalities
Fetus - metabolism
Fetus - pathology
gastrin-releasing peptide
Gastrin-Releasing Peptide - metabolism
Glycoproteins - metabolism
Humans
Hyaluronan Receptors - metabolism
lung
Medical sciences
Neoplasm Proteins - metabolism
Peptides - metabolism
Pneumology
Protein Precursors - metabolism
Proteolipids - metabolism
Pulmonary Surfactant-Associated Protein C
Pulmonary Surfactants - metabolism
Respiratory system : syndromes and miscellaneous diseases
Surface-Active Agents - metabolism
surfactant
type 1 cell-associated antigen
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Summary:Congenital cystic adenomatoid malformation of the lung (CCAM) is a rare congenital lesion whose pathogenesis is not well defined. It is generally accepted that the various types of CCAMs originate at different levels of the tracheobronchial tree. To further define the pathogenesis of CCAM, we evaluated the cellular composition of different CCAM types by immunohistochemistry. Twenty-two CCAMs (17 CCAM type 1, two type 2, one type 3, and two type 4) were collected. The cellular composition was determined using immunohistochemical stains for type I cell-associated antigen (T 1 cell-Ag), surfactant proteins and surfactant protein precursors (SP-A, SP-B, proSP-B, and proSP-C), neuroendocrine cells (GRP), Clara cells (UP-1), and the adhesion molecule CD44v6, a glycoprotein thought to be involved in cell-matrix and cell-cell interactions. Eleven fetal lungs also were analyzed to compare cytodifferentiation of the epithelial-lined cysts of the different types of CCAM with the stages of normal lung development. Our results indicate that CCAM is caused by an arrest in lung development, and, on the basis of cytodifferentiation, two major subtypes can be distinguished. One subtype consisting of CCAM types 1, 2, and 3 that shows a bronchiolar type of epithelium and a second subtype, consisting of CCAM type 4, that has an acinar-alveolar type of epithelium. Our findings also suggest that these two subtypes may arise at different stages of the branching of the bronchopulmonary tree, the first at the pseudoglandular stage and the second at the saccular stage.
ISSN:0046-8177
1532-8392
DOI:10.1016/S0046-8177(99)90084-9