Pulmonary congenital cystic disease in adults. Spiral computed tomography findings with pathologic correlation and management

Abstract

Purpose: The aim of this study was to assess the computed tomography (CT) features of intrapulmonary congenital cystic diseases in adults and to correlate the imaging features with the pathological findings, with emphasis on the oncogenic potential of the lesions.

Materials and methods: We retrospectively reviewed the CT scans in three institutions from August 1996 to December 2008, of nine patients (six men, three women; mean age 48.6 years; range 26-75 years) who had histological diagnosis of pulmonary cystic disease after surgery. Six patients had a diagnosis of intrapulmonary bronchogenic cyst (IBC), and three had a type-I cystic adenomatoid malformation (CAM). In one case, intralobar sequestration (ILS) was associated with type-I CAM.

Results: Three patients were symptomatic and six were asymptomatic. On CT scans, IBCs showed homogeneous fluid attenuation (n=2), air-fluid level (n=2), air attenuation (n=1) or soft-tissue attenuation (n=1). The surrounding lung tissue showed areas of band-like linear attenuation in three IBCs, atelectasia in two and mucocele-like areas in one. On CT, type-I CAM appeared as a unilocular cystic lesion with air-fluid level (n=1) or air content (n=1). Both cases had thin walls surrounded by normal lung parenchyma. ILS appeared as a fluid-filled cyst with afferent and efferent vessels. Of the six IBCs, one occurred in the upper right lobe, two in the middle lobe and three in the lower right lobe. Of the three type-I CAMs, one was in the upper left lobe and one in the middle lobe. The type-I CAM associated with ILS was located in the left lower lobe.

Conclusions: The similar CT patterns preclude differentiation between IBC and type-I CAM. Surgical resection of all intrapulmonary cystic lesions detected in adults is mandatory because type-I CAM is a precursor of mucinous bronchioloalveolar carcinoma.