Tuberc Respir Dis.  2008 Aug;65(2):110-115.

Congenital Cystic Adenomatoid Malformation of Lung in Adults: Clinical, Pathologic and Radiologic Evaluation of Six Patients

• Park YJ
• Jung H
• Park IN
• Choi SB
• Hur JW
• Lee HP
• Yum HK
• Choi SJ
• Koo HS
• Lee YH
• Choi SJ
• Jung SJ
• Lee HK
• Kim AR

Abstract

BACKGROUND: Congenital cystic adenomatoid malformation of the lung (CCAM) is a rare congenital developmental anomaly of the lower respiratory tract. Most cases are diagnosed within the first 2 years of life, so adult presentation of CCAM is rare. We describe here six adult cases of CCAM and the patients underwent surgical resection, and all these patients were seen during a five and a half year period. The purpose of this study was to analyze the clinical, radiological and histological characteristics of adult patients with CCAM.
METHODS
Through medical records analysis, we retrospectively reviewed the clinical characteristics, the chest pictures (X-ray and CT) and the histological characteristics.
RESULTS
Four patients were women and the mean age at diagnosis was 23.5 years (range: 18~39 years). The major clinical presentations were lower respiratory tract infection, hemoptysis and pneumothorax. According to the chest CT scan, 5 patients had multiseptated cystic lesions with air fluid levels and one patient had multiple cavitary lesions with air fluid levels, and these lesions were surrounded by poorly defined opacities at the right upper lobe. All the patients were treated with surgical resection. 5 patients underwent open lobectomy and one patient underwent VATS lobectomy. On the pathological examination, 3 were found to be CCAM type I and 3 patients were CCAM type II, according to Stocker's classification. There was no associated malignancy on the histological studies of the surgical specimens.
CONCLUSION
As CCAM can cause various respiratory complications and malignant changes, and the risks associated with surgery are extremely low, those patients who are suspected of having or who are diagnosed with CCAM should go through surgical treatment for making the correct diagnosis and administering appropriate treatment.

Keyword

Adult; Congenital cystic adenomatoid malformation; Surgery

MeSH Terms

Adult
Cystic Adenomatoid Malformation of Lung, Congenital
Female
Hemoptysis
Humans
Lung
Medical Records
Pneumothorax
Respiratory System
Respiratory Tract Infections
Retrospective Studies
Thoracic Surgery, Video-Assisted
Thorax

Figure

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  Figure 1 There are multiple cystic lesions, about 6~7 cm in diameter in the left middle lung field. Pneumonic consolidations and air fluid levels are in and around the cystic lesions (A). At the level of the left inferior pulmonary vein, the lesion contains about 4.5 cm in diameter cystic space (B). The CT scan at the level of left main bronchus reveals innumerable fine cystic lesions in the superior segment of the left lower lobe (C).

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  Figure 2 Chest PA shows a cystic lesion with air-fluid level and pericystic pneumonic infiltration in right lower lobe on admission (A). Chest CT scan on admission reveals a thin walled multicystic lesion with fluid collection mimics a pulmonary abscess in RLL (B).

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  Figure 3 Gross finding of a resected right lower lobe in Case 6 shows one large cystic lesion and small variable sized cysts surrounding by bronchial like structures, accompanied with grayish pneumonic consolidation.

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  Figure 4 The mass is composed of multiple, variable sized cysts in Case 3 (CCAM Type 1) (A, H&E stain, ×40). The cysts are lined by ciliated pseudostratified columnar epithelium with frequent mucus producing cells. The wall of the cysts contains cartilage (B, H&E stain, ×200).

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