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Abstract


Congenital cystic adenomatoid malformation (CCAM) of the lung is a rarest form of
the congenital pulmonary cystic disease. This lesion was initially introduced in the
English literature by Chin & Tang in 1949. Thereafter, many other cases were reported.
In Korean literatures nine cases were reported previously.
Recently we have experienced a case of CCAM born with generalized edema
associated with maternal hydroamnios. This premature baby was delivered at 30 weeks
of gestation.
At autopsy, right lower lung showed a large cystic mass which had a connection to
the bronchus. This mass compressed the superior vena java and the right atrium.
Microscopically the cystic mass was characterized by coarsely trabeculated pattern with
numerous cystic spaces that were lined by respiratory epithelium or tall columnar
epithelium. Gland-like spaces or alveolar spaces that are lined by simple cuboidal or
columnar epithelium with occasional mucus-producing cells were also noted. Bronchial
mucous gland and cartilage were not found. Other lobes of lung and the remaining
organs did not show any specific anomalies.

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