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KMID : 0357919860200020215
Korean Journal of Pathology
1986 Volume.20 No. 2 p.215 ~ p.221
Congenital mystic Disease of the Lung


Abstract
Congenital cyst of the lung is a type of rare pulmonary disease. This lesion was
initially introduced by Morgagni in 1769. Thereafter many other cases were reported.
Etiology or developemental pathogenesis of this lesion is not certain, but abnormal
developement of embryonic lung bud during the embryonal period is highly suspected.
This lesion is two times more frequently located at the left lung, but both sides of the
lung may be involved. This lesion is largely asymptomatic and found incidentally but
can be evoked respiratory symptoms and also can be died due to only this lesion. So
accurate diagnosis and treatment in the early period are very important. Therefore, 32
cases of congenital cyst of the lung reported at the department of pathology, seoul
national university hospital from 1. 1968 to 12. 1984 were reviewed and classified with
clinical, gross and microscopic findings based on the Buntain's classification. Among 32
cases of congenital cyst of the lung, 16 cases were bronchogenic cysts and were largest
in number. 8 cases were pulmonary sequestration, 6 cases were congenital cystic
adenomatoid malformation (CCAM) and 2 cases were pulmonary emphysema. In cases of
bronchogenic cyst, involved age was variabe from 7 months to 51 years and large cases
were asymptomatic. Grossly, 15 cases were solitary cyst and only one case was
multiple. Size was also variable from 3 cm to 11.5 cm. Microscopically, cyst wall was
lined by pseudostratified ciliated columnar epithelium with gobet cells, mucous secreting
glands, smooth muscle fibers and fibrous interstitial tissue. Three cases were also
exhibit cartilage at the wall.
In cases of CCAM, there were involved below the 7 months old boys and girls. Four
cases were confirmed at the autopsy, other 2 cases were confirmed at the surgical
specimens which were found incidentally or due to generalized cyanosis. Microscopic
findings were similar in all cases showing relatively well demarcated multiple small
cysts with adenomatous proliferation. Lining epithelial cells were tall columnar,
pseudostratified ciliated columnar epithlium with occasional mucous secretion. Stroma
also exhibit adenomatous proliferation lined by simple columnar or cuboidal epithelium.
In cases of pulmonary sequestration, 7 cases were intrapulmonary, other one case was
extrapulmonary sequestration. In five cases, systemic large vessel were confirmed.
Microscopically it was composed of irregularly dilated bronchiolar structures lined by
pseudostratified ciliated columnar epithelium.
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