성인에서 진단된 선천성 낭포성 유선종 폐기형 6예 = Congenital Cystic Adenomatoid Malformation of Lung in Adults: Clinical, Pathologic and Radiologic Evaluation of Six Patients

국문 초록 (Abstract)

연구배경: CCAM은 드문 하부 호흡기의 선천성 발달 기형으로 대부분은 생후 2년 내에 진단되는 것으로 보고되고 있다. 그리고 매우 드물게 성인이 되어서 호흡기 합병증이 생기거나 무증상인 채로 우연히 진단된 증례들이 보고되었다. 우리는 본원에서 수술을 통해 조직학적 소견으로 확진된 6명의 성인 CCAM 환자들을 기술하였고 이들의 임상적, 방사선학적 및 조직학적 특성을 분석하고자 하였다. 방법: 2001년 8월부터 2007년 2월까지 본원에서 성인기에 수술을 통한 조직학적 소견으로 확진된 6명의 CCAM 환자를 확인하였다. 의무기록 검토를 통해 그들의 인구학적 및 임상적 특성, 흉부 방사선 자료 그리고 조직학적 특성을 후향적으로 분석하였다. 결과: 6명의 CCAM 환자 중 4명이 여자였고 진단 당시의 평균 나이는 23.5세(범위 18∼39세)였다. 내원 당시 주요 임상 양상은 하부 호흡기 감염, 객혈, 그리고 기흉이었다. 흉부 전산화 단층촬영을 보면 5명의 환자는 공기 액체층을 동반한 다발성 격막을 가진 낭종성 병변을 가지고 있었고 나머지 1명에서는 우상엽에 공기 액체층을 동반한 다발성 공동성 병변과 주위에 다발성 침윤성 병변을 가지고 있었다. 모든 환자는 폐엽 절제 수술을 받았는데 5명은 개흉술을 통한 폐엽 절제술을 그리고 1명은 흉강경을 이용한 폐엽 절제술을 시행 받았다. 조직학적 검사결과 Stocker씨 분류법에 따라 3명의 환자는 1형 그리고 나머지 3명은 2형으로 분류되었다. 모든 환자의 검체에서 동반된 악성 종양의 증거는 없었다. 결론: CCAM이 하부 호흡기 감염, 기흉, 객혈 등의 다양한 호흡기 합병증과 악성화를 일으킬 가능성이 있고 수술적 치료와 연관된 합병증이 거의 없기 때문에 CCAM으로 의심되거나 확실치 않은 폐의 낭종성 혹은 공동성 병변이 있을 때 정확한 진단과 적절한 치료를 위해서 반드시 수술적 치료가 필요하다.

다국어 초록 (Multilingual 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. (Tuberc Respir Dis 2008;65:110-115)

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