• Journal of Chest Surgery
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• v.28 no.7
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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.

• Tuberculosis and Respiratory Diseases
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• v.55 no.1
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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.

• Advances in pediatric surgery
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• v.2 no.2
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• pp.138-142
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• 1996

Pulmonary sequestration is a complex anomaly involving the pulmonary parenchymal tissue and its vasculature. It presents as a cystic mass of nonfunctional lung tissue without communication with the tracheobronchial system. Usually, it receives blood supply from anomalous systemic vessels. Therefore, preoperative diagnosis of the pulmonary sequestration is difficult, especially when it is located in the abdomen and combined with congenital cystic adenomatoid malformation(CCAM). We encountered such a mass(CCAM type 2) detected prenatally by ultrasonography. It was a kidney bean shaped, pinkish mass straddling the thorax and abdomen on the right side. Because of the sonographic appearance, neuroblastoma was diagnosed preoperatively. The mass was completely extirpated without difficulty.

• Journal of Chest Surgery
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• v.26 no.4
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• pp.320-324
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• 1993

Congenital cystic adenomatoid malformation [CCAM] of the lung is extremely rare. The patient was 10 year old female and had no specipic signs and symptoms except right lower chest pain for 5 days ago before admission.On simple chest X-ray and thoracic CT scan, about 9x8x8cm sized, heterogenous marginal enhanced multiseparated hypodence lesion with air fluid level and gas bubbles in posterior pleural space in right lower chest .The culture result of needle aspiration of cavity was apergillus flavus. Right lower lobectomy was carried out and the result of biopsy was congenital cystic adenomatoid malformation.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.805-811
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• 1996

Congenital cystic adenomatoid malformation of the Lung(CCAM) is characterized by anomalous fetal development of terminal respiratory structures, resulting in an adenomatoid proliferation of bronchiolar elements and cystic formation. CCAM was first described and differentiated from other cystic lung disease in the English literature by Ch'in and Tang in 1949. CCAN is a rare, potentially lethal form of congenital pulmonary cystic disease and the salient features of lesion are an irregular network of terminal respiratory bronchiole-like structures and macrocysts variably lined by pseudostratified ciliated columnar epithelium and simple cuboidal epithelium. Adult presentation of CCAM of the lung is so rare that only 9 cases have been reported in the literature of date. The pathogenesis of CCAM remains disputed and reseachers have variously proposed that the lesion represents a developmental anomaly, hamartoma, or a fonn of pulmonary dysplasia. Van Dijk and Wagenvoort divided CCAM into three subtypes : cystic, intermediated, and solid. These correspond to types I, II, and III of Stocker. In adults, the evaluation of cystic or multi cystic lung disease requires consideration of a differential diagnosis including the acquired lesions of lung abscess, cavitary neoplasm or inflammatory mass, bullous disease, bronchiectasis, and postionflammatory pneumatocele. Congenital lesions such as sequestration, bronchopulmonary-foregut anomalies, and bronchogenic cyst are also encounted. The definitive treatment for CCAM is complele removal of the involved lobe. Panial lobectomy leads to multiple complications, including severe post-operative infection. We report a case of CCAM in a 14-year-old female presentated with a pneumothorax and large bullae, who was treated by surgical remove of the involved lobe.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.352-357
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• 1986

Congenital cystic adenomatoid malformation [CCAM] of the lung is a rare but often fatal congenital lung bud anomaly that causes acute respiratory distress in the newborn infants. In those who survive infancy and early childhood, its clinical manifestations are not unusual to detect. Recently we experienced two children with CCAM; one with coughing and high fever, another without any symptom On case I, the patient was a 9 year-old female, and was suffered from coughing and high fever since 15 days before. Medical treatment was failed and then, exploratory thoracotomy was performed under the impression of loculated empyema. Finally, a right lower lobectomy was done with a satisfactory postoperative course. On case II, the patient was a 12 year-old female, and admitted for evaluation of known large pulmonary cyst which was checked at the outpatient clinic. Preoperative evaluations gave no more informations, so obliteration of cysts was performed with open biopsy. Two patients with CCAM are described in Korean Univ. Medical Center.

• Tuberculosis and Respiratory Diseases
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• v.74 no.1
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• pp.28-31
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• 2013

We present a case of congenital cystic adenomatoid malformation (CCAM) in a 25-year-old male who was presented with chronic cough. Chest radiography revealed an abnormal mass-like shadow in the right lower pulmonary zone. A contrast enhanced computed tomography showed an 11 cm solid, cystic mixed mass on the right lower lobe. A right lower lobectomy was performed by video-assisted thoracoscopic surgery without complications. The gross specimen showed a massive cavitation with multiloculated cysts of varying size, consistent with CCAM, along with noticeable granulomatous inflammation. Non-tuberculosis mycobacteria were isolated from a bronchial wash specimen, and the resected tissue homogenates were positive for Mycobacterium avium-intracellulare complex by polymerase chain reaction.

• Tuberculosis and Respiratory Diseases
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• v.72 no.6
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• pp.501-506
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• 2012

Congenital cystic adenomatoid malformation (CCAM) is an uncommon, nonhereditary anomaly caused by arrest of lung. Patients with CCAM may present with respiratory distress as newborns, or may remain asymptomatic until later in life. CCAM type I is rarely found in association with bronchial atresia (BA) in adults; we present such a case. Case: A 54-year-old female presented with chronic cough and blood-tinged sputum. Physical examination and laboratory tests were unremarkable. Chest radiographs and a CT scan of the chest showed multiple large air-filled cysts consistent with a CCAM in the right lower lobe, and an oval-shaped opacity in the distal right middle lobal bronchus. Based on the radiologic findings, right middle lobectomy and a medial basal segmentectomy of the right lower lobe were performed via a thoracotomy. These lesions were consistent with Stocker's Type I CCAM and BA in the different lobes.

• Tuberculosis and Respiratory Diseases
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• v.66 no.5
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• pp.385-389
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• 2009

Congenital cystic adenomatoid malformation (CCAM), which is classified into five types according to size and bronchial invasion, is a rare type of developmental anomaly of the lung. CCAM is occasionally accompanied by malignancy, such as bronchioloalveolar carcinoma (BAC) or rhabdomyosarcoma. As defined by the WHO, atypical adenomatous hyperplasia (AAH) is a non-invasive spread of atypical epithelial cells in single rows along the alveolar wall, within a lesion that is usually less than 5 mm in diameter. AAH was also regarded as a pre-invasive neoplasia, especially associated with BAC and adenocarcinoma. We report a case of type II CCAM with AAH in adults, with a review of the references.

• Journal of Chest Surgery
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• v.21 no.6
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• pp.1095-1098
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• 1988

Congenital cystic adenomatoid malformation[CCAM] is a rare disease that cause respiratory distress in the newborn and infants, but is one of the two causes along with lobar emphysema. This malformation has the pathologic characteristics which can be differentiated from other forms of diffuse cystic disease, i.e. CCAM is marked proliferation of the terminal bronchioles and that can enlarge rapidly by air trapping in cystic areas. The CCAM has a clinical importance because of rapid worsening respiratory distress, with tachypnea, subcostal retraction and cyanosis. This is a strict surgical condition and after operation[lobar, segmental resection or pneumonectomy] the symptoms relieved obviously. We experienced 6 cases of CCAM from July, 1980 to September, 1987 at the Department of Cardiovascular and Thoracic Surgery, Severance Hospital, College of Medicine, Yonsei University. The male patients were two and female patients were four. The age distribution was from premature to 10 year old. One of them was 27 weeks gestational premature female who was borne dead. The other 5 patients were performed on thoractomy[1 case pneumonectomy and 5 cases lobectomy]. The postoperative courses were good and no complications were seen.