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Congenital pulmonary airway malformation
Congenital Pulmonary Airway MalformationCPAM
Formerly known as Congenital Cystic Adenomatoid MalformationCCAM
The former designation “CCAM” is changed to “CPAM” because the lesions are
“Cystic” in only three of the five types
and “adenomatoid” in only one type (type 3).
CPAM more accurately encompasses all the 5 types
CPAM is relatively rare, affecting about 1 in 25,000 -35,000 pregnancies though rare overall, it is the most common type of congenital lung lesion (update 2021)
It is slightly more common in males than in females.
CPAM is not hereditary, so it usually does not occur in families.
There are no predictive factors such as sex, ethnicity, or maternal age at delivery.
Replacement of a portion of the lung by the nonfunctioning cystic lesion.
It can affect any lobe of the lung
It doesn’t seem to be an area that is more likely to be affected than another.
There is no known genetic predisposition, with the exception of type 4 malformations,
which have been associated with a familial pleuropulmonary blastoma (PPB) syndrome
Many of patients with Familial Pleuropulmonary blastoma (PPB)
have a mutation of the DICER 1 gene.
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