• Journal of Chest Surgery
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• 제28권7호
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• pp.726-730
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• 1995

Congenital cystic adenomatoid malformation[CCAM of the lung is extremely rare. We have experienced an unusual case of congenital cystic adenomatoid malformation. The patient was 20-year-old male and had chest pain for 10 days. On simple chest x-ray and Thoracic CT scan, there was a large cystic mass surrounded with multiloculated round cysts with air fluid level on the right lower lobe of a lung. Right lower lobectomy was performed and the pathologic result was congenital cystic adenomatoid malformation.

• Journal of Chest Surgery
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• 제24권8호
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• pp.819-823
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• 1991

The congenital cystic adenomatoid malformation of the lung is a rare disease, and is one of the most common congenital lung diseases which require prompt surgical intervention. The prognosis depends on its tissue type, prompt diagnosis and surgical intervention. The lesion consists of enlarged, variable sized multiple cyst with overgrowth of terminal bronchioles, like hamartoma. This disease can be associated with other vascular anomalies or other congenital defect especially in type II lesion We recently experienced one case of congenital cystic adenomatoid malformation The patient was 2 months old infant who showed respiratory distress without associated anomaly. After right upper lobe lobectomy, the patient was recovered uneventfully.

• Tuberculosis and Respiratory Diseases
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• 제55권1호
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• pp.107-112
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• 2003

A congenital cystic adenoid malformation of the lung(CCAM) is characterized by an anomalous fetal development of the terminal respiratory structures, resulting in the adenomatoid proliferation of the bronchiolar elements and cystic formation. CCAM has been detected on the fetus, premature babies and stillborn as well as infants and children. An adult presentation of CCAM is extremely rare. When cystic lesions occur with a repeated infection, an evaluation of the cystic lesions requires a differential diagnosis of CCAM, sequestration, a lung abscess, a pneumatocele and a bronchogenic cyst. The definite treatment of CCAM is the surgical removal of the involved lobe. We report a case of a CCAM in a 24-year-old female with a brief review of the relevant literature.

• Clinical and Experimental Pediatrics
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• 제61권1호
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• pp.30-34
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• 2018

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 ($GGT{\rightarrow}GAT$), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM.

• Anatomy and Cell Biology
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• 제55권2호
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• pp.264-268
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• 2022

Congenital cystic adenomatoid malformation (CCAM) is a rare developmental dysplastic lesion that affects the fetal bronchial tree. Etiopathogenesis is still poorly understood. Most accepted view is that of abnormal branching of bronchioles during the period of morphogenesis. We observed a rare congenital anomaly of the lungs during fetal autopsy. Routine antenatal ultrasonography revealed multiple echolucent cysts in the right lung of the fetus. Thorough external and internal examination was followed by sectioning of each organ for histopathological examination. Histopathology of the right lung showed distortion of the parenchyma with dilated bronchioles. Multiple cysts lined by columnar epithelium along with loose intervening connective tissue were observed along with many congested and dilated blood vessels. Knowledge of congenital anomalies of the respiratory system would help clinicians to plan the management at a very early stage. Accurate fetal autopsy along with clinical data is important in evaluating fetal deaths and can help in reducing unexplained stillbirths.

• Journal of Chest Surgery
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• 제13권3호
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• pp.292-297
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• 1980

The congenital cystic adenomatoid malformation of the lung consists of an enlarged, meaty, multicystic lobe with smooth-walled cysts of varying sizes, which can communicate with major bronchi through malformed air passages that usually lacks cartilage. This abnormality is usually symptomatic in infancy with signs of respiratory distress such as tachypnea, substernal retraction and cyanosis. Prompt surgical resection is choice of treatment in life-threatening respiratory distress patients. We recently experienced a case of congenital cystic adenomatoid malformation of the lung in a patient with situs inversus totalis. The patient was 40 days old female who showed severe respiratory difficulty. Emergency left middle lobectomy was undergone successfully. Her postoperative course was uneventful. She was discharged from hospital on the postoperative eighth day in good condition.

• Journal of Chest Surgery
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• 제49권3호
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• pp.210-213
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• 2016

Congenital cystic adenomatoid malformation is a rare, but well-known disease. It can be managed conservatively in patients without symptoms or require surgical removal when symptomatic. The surgical option of choice is en bloc resection of the affected lesion. We report an experience of life-threatening congenital cystic adenoid malformation in a low-birth-weight (1,590 g) premature neonate who was successfully treated with a lobectomy of the lung.

• Clinical and Experimental Pediatrics
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• 제49권7호
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• pp.796-799
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• 2006

편측성 다낭성 신 이형성증과 폐의 선천성 낭성 선종양 기형은 각각 독립된 질환으로 배아 시기에 발생 과정의 문제로 생긴다고 알려져 있다. 폐의 선천성 낭성 선종양 기형에서 폐 외의 기형으로 신장 기형이 동반될 수 있으나 생존 가능성이 적어 사산아로 출생되는 경우가 많다. 저자들은 위장관염으로 입원한 21개월 남아에서 우연히 편측성 다낭성 신 이형성증을 발견했고, 이 과정에서 폐의 선천성 낭성 선종양 기형도 발견하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제40권11호
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• pp.793-797
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• 2007

Communicating bronchopulmonary foregut malformation (CBPFM)은 매우 드문 선천성 기형으로 격리된 호흡계 조직과 식도 또는 위장 사이의 누관이 특징적이다. 원시위장관에서 기원한 부속의 폐조직이 모든 종류의 bronchopulmonary furegut malformation 발생의 중요한 요인이 된다. 반복되는 폐렴이 있는 환자의 chest X-ray에서 낭성 병변이 보인다면 CBPFM을 생각해봐야 하며, 진단적 방법으로 식도조영술, 기관조영술, 컴퓨터 단층촬영, 자기공명영상 그리고 혈관조영술 등이 도움이 된다. 치료는 수술적 제거가 권장되며 예후도 좋다. 좌측 완전 심낭결손증과 동반되었으며, 이중식도에 연결된 외엽성 폐격리증과 기관지성 낭종을 보인 CBPFM 1예를 보고한다.

• Journal of Chest Surgery
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• 제33권7호
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• pp.594-596
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• 2000

선천성 낭종성 선종양 기형과 외엽형 폐격리증은 아주 드문 질환이다. 본원에서는 4세 된 여자환자에서 좌폐화엽의 선천성 낭종성 선종양 기형을 수술하던 중 우연히 외엽형 폐격리증이 동반된 것을 알고 좌폐하엽 절제술과 외엽형 폐격리증 절제술을 시행하였는데, 외엽형 폐격리증은 흉부대동맥에서 비정상적으로 직접 1개의 동맥으로 유입되고, 늑간정맥을 통하여 기정맥으로 유출되었다. 환자는 수술 후 건강하게 퇴원하였다.