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Park, Jung, Park, Choi, Hur, Lee, Yum, Choi, Koo, Lee, Choi, Jung, Lee, and Kim: Congenital Cystic Adenomatoid Malformation of Lung in Adults: Clinical, Pathologic and Radiologic Evaluation of Six Patients
Original Article
Tuberculosis and Respiratory Diseases

Tuberculosis and Respiratory Diseases 2008; 65(2): 110-115.

Published online: 30 August 2008

DOI: https://doi.org/10.4046/trd.2008.65.2.110

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

Young Jin Park, M.D.1, Hoon Jung, M.D.1, I-Nae Park, M.D.1, Sang Bong Choi, M.D.1, Jin-Won Hur, M.D.1, Hyuk Pyo Lee, M.D.1, Ho-Kee Yum, M.D.1, Soo Jeon Choi, M.D.1, Ho-Seok Koo, M.D.1, Yang-Haeng Lee, M.D.2, Suk-Jin Choi, M.D.3, Soo-Jin Jung, M.D.4, Hyun-Kyung Lee, M.D.1, Ae Ran Kim, M.D.5

1Department of Internal Medicine, College of Medicine, Inje University, Busan, Korea.

2Department of Thoracic Surgery, College of Medicine, Inje University, Busan, Korea.

3Department of Radiology, College of Medicine, Inje University, Busan, Korea.

4Department of Pathology, College of Medicine, Inje University, Busan, Korea.

5Department of Internal Medicine, Daedong Hospital, Busan, Korea.

Address for correspondence: Hyun-Kyung Lee, M.D. Department of Internal Medicine, Pusan Paik Hospital, Inje University College of Medicine, 633-165, Gae-gum 2-dong, Jin-gu, Busan 614-735, Korea. Phone: 82-51-890-6270, Fax: 82-51-892-0273, goodoc@gmail.com

Received 17 June 2008       Accepted 24 July 2008

Copyright © 2008 The Korean Academy of Tuberculosis and Respiratory Diseases

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.

Keywords: Adult, Congenital cystic adenomatoid malformation, Surgery

Figures and Tables

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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Table 1
Clinical and histologic data for six adult patients with congenital cystic adenomatoid malformation of lung
trd-65-110-i001

VATS: video assisted thoracoscopic surgery.

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