Korean J Pathol.  1986 Jun;20(2):215-221.

Congenital Cystic Disease of the Lung

• Sohn JH
• Chi JG

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. 1986 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 variable 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 epithelium 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.

MeSH Terms

Cysts