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Pathology of Congenital Cystic Adenomatoid Malformation

Dr Sampurna Roy MD

 

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare but potentially life-threatening pulmonary anomaly.

Congenital cystic adenomatoid malformation (CCAM) can present at different ages and in various clinical presentations.

There can even be asymptomatic patients who receive their diagnosis during the prenatal period.

The possibility of an underlying Congenital cystic adenomatoid malformation should be considered in infants with recurrent chest infections or persistent abnormalities on chest X-ray following an acute infection.

Congenital cystic adenomatoid malformation should also be considered in cases with lung abscess, even in adolescents with no past history of pulmonary complaints.

This congenital malformation features abnormal air-spaces lined by cuboidal and columnar epithelium reminiscent of the lining of bronchi and bronchioles. 

The air-spaces range from a complex of a few large cysts to innumerable tiny spaces resembling developing lung.

Small amounts of cartilage and groups of goblet cells may be seen in association with large cysts.

The cystic adenomatoid malformation of the lung is an hamartomatous lesion , easily identifiable by its morphology through the application of Stocker's et al (1977) classification (type 1, 2 and 3) and also following the criteria of Yousem, to understand the five types dependent on the level of malformation in the airway and lung.

The three morphological types are as follows:

Type I      Large cyst type 65%    

Type II     Small cyst type 25%    

Type III    Solid type 10%

Presentation is usually in infancy with respiratory distress or recurrent chest infections but cases detected in adult life are described.

Surgical resection is the standard treatment in a disease that is almost always unilateral and unilobar.

The mucus cell hyperplasia seen in the type I malformation may provide the substrate for the subsequent development of carcinomas.

The type II lesion is associated with other congenital malformations such as renal dysgenesis and diaphragmatic herniae.

The differential diagnosis of type I lesion includes intralobar sequestration, bronchogenic cyst and bronchial atresia while lesions with smaller cysts have to be distinguished from congenital lymphangiectasia, interstitial emphysema and congenital lobar emphysema.

 

Further reading:

Cystic adenomatoid malformation of the lung in an adult.

Clinicopathologic assessment of prenatally diagnosed lung diseases.

Prenatal period to adolescence: the variable presentations of congenital cystic adenomatoid malformation.

Congenital cystic adenomatoid malformation associated with ipsilateral eventration of the diaphragm.

Congenital cystic adenomatoid malformation in the fetus: a hypothesis of its development.

Sequestrations, congenital cystic adenomatoid malformations, and congenital lobar emphysema.

Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not?

Congenital cystic adenomatoid malformation of the lung or congenital pulmonary airway malformation.

Congenital cystic adenomatoid malformation in the newborn: two case studies and review of the literature.

Congenital cystic adenomatoid malformation of the lung. Analysis of 10 cases and a review of literature.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 


 

 

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