TY - JOUR
T1 - Congenital cystic adenomatoid malformation of the lung (CCAM)
T2 - Evaluation of the cellular components
AU - Morotti, Raffaella A.
AU - Cangiarella, Joan
AU - Gutierrez, Maria C.
AU - Jagirdar, Jaishree
AU - Askin, Fred
AU - Singh, Gurmukh
AU - Profitt, Sherri A.
AU - Wert, Susan E.
AU - Whitsett, Jeffrey A.
AU - Greco, M. Alba
N1 - Funding Information:
Supported in part by NIH IlL 56387. Specialized Center of Research in Pathobiology of Lung Development, awarded to S.E.W. andJ.A.W.
PY - 1999/6
Y1 - 1999/6
N2 - 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 (T1 cell-Ag), surfactant proteins and surfactant protein precursors (SPA, 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 CGAM 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 CGAM 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.
AB - 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 (T1 cell-Ag), surfactant proteins and surfactant protein precursors (SPA, 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 CGAM 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 CGAM 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.
KW - Clara cell antigen
KW - Congenital cystic adenomatoid malformation
KW - Gastrin- releasing peptide
KW - Lung
KW - Surfactant
KW - Type 1 cell-associated antigen
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U2 - 10.1016/S0046-8177(99)90084-9
DO - 10.1016/S0046-8177(99)90084-9
M3 - Article
C2 - 10374767
AN - SCOPUS:13044256414
SN - 0046-8177
VL - 30
SP - 618
EP - 625
JO - Human pathology
JF - Human pathology
IS - 6
ER -