• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.13 no.1
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• pp.139-143
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• 1983

A 18-year-old man had a painless swelling in the right anterior portion of maxilla for 2 years. On radiographic examination, a radiolucent region that was not associated with an unerupted tooth was seen. Small scattered radiopaque foci were seen in the cystic lumen. At second case, a 16-year-old girl had a painless swelling in the anterior portion of maxilla for 3 years. On radiographic examination, a radiolucent region that associated with an unerupted tooth was seen. Multiple scattered radiopaque foci were seen in the radiolucent cystic lumen. With the patient under local anesthesia, well encapsulated tumors were enucleated. The diagnosis made in the pathologist's report was Adenomatoid Odontogenic Tumor, benign lesion often having distinct clinical and radiographic features.

• Journal of Chest Surgery
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• v.18 no.4
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• pp.785-791
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• 1985

The congenital cystic adenomatoid malformation [C.C.A.M.] of the lung is a rare lesion consisted of multiple cysts in the pulmonary parenchyma lined cuboidal or columnar epithelium. The C.C.A.M. presents clinically in three ways:[1] stillborn or perinatal death, [2] progressive respiratory distress in the newborn, and [3] acute and chronic pulmonary nfections in the older infant and child. We recently experienced two cases of C.C.A.M. of the lung, and have good surgical results. The first case was 29 days old male and the second case was 16 month old female who have been suffered from severe respiratory difficulty and pulmonary infection repeatedly each other. The lobectomies were performed, and the postoperative course was uneventful.

• 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.17 no.1
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• pp.118-124
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• 1984

Congenital Cystic Adenomatiod Malformation (C.C.A.M.) is rare, but one of the most common congenital pulmonary anomalies that cause acute respiratory distress in the newborn infants. It is characterized and differentiated from the diffuse pulmonary cystic disease pathologically, i.e. adenomatoid appearance due to marked proliferation of the terminal respiratory components. An 2/12 year old male patient was suffered from respiratory distress and cyanosis on crying since birth, but no specific therapy was given. With progression of symptoms, he came to Korea University Hospital for further evaluation and then transfered to Dept. of Chest Surgery for operative correction under the impression of Congenital Obstructive Emphysema suggested by a pediatrician. On gestational and family history, there was nothing to be concerned such as congenital anomaly. Physical examinations showed; moderate nourishment and development (Wt. 5.5kg), cyanosis on crying, both intercostal and lower sternal retraction on inspiration, Lt. chest building with tympany, Rt. shifting of cardiac dullness, decreased breathing sound with expiratory wheezing on entire Lt. lung field, decreased breathing sound on Rt. upper lung filed, and tachycardia. The remainders were nonspecific. Laboratory findings were normal except WBC $14000/mm^3$ (lymphocyte 70%), Hgb 9.8m%, Hct 28%, negative Mantaux test, and sinus tachycardia and counter-clockwise rotation on EKG. Preoperative simple Chest PA revealed marked hyperlucent entire Lt. lung, herniation of Lt. upper lobe to Rt., collapsed Rt. upper lobe, tracheal deviation and mediastinal shifting to Rt., and no pleural reaction. At operation, after Lt. posterolateral thoracotomy, 4th rib was resected. Operative findings were severe emphysematous changes limited to both lingular segmentectomy was done. The resected specimen showed slight solidity, measuring $8{\times}4.5{\times}2cm$ in size, and small multiple cystic spaces filled with air. Microscopically, entire tissue structures were glandular in appearance, cyst were lined by ciliated columnar epithelium, and occasional cartilages were noted around the cystic spaces. Bronchial elements were dilated but normal pattern on histologically. The patient had a good postoperative courses clinically and radiologically, and discharged on POD 10th without event. The authors report a case of Cogenital Cystic Adenomatoid Malformation (C.C.A.M.)

• Journal of Chest Surgery
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• v.23 no.4
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• pp.698-706
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• 1990

Congenital Cystic Lung Disease is a spectrum of closed related anomalies that arise during an early stage of embryonic lung bud maturation-namely bronchogenic cyst, congenital lobar emphysema, pulmonary sequestration and congenital cystic adenomatoid malformation. And they show similar surgical strategies. So they are called as the term bronchopulmonary-foregut malformations, firstly proposed by Gerle[1968]. From Aug. 1979 to Aug 1989, 47 patients were operated upon on Dept. of Thoracic & Cardiovascular Surgery at the CUMC. There were 21 females and 26 males ranging in age from age of 21 day to age of 56 year [15 cases under 15 years old]. 30 patients had bronchogenic cysts - 23 of intrapulmonary type, 7 of mediastinal type in location. Affected lobes and locations were as follows: 11 in upper lobe, 3 in middle lobe, 11 in lower lobe and anterosuperior, middle, and posterior mediastinal type were 3, 2, 2 respectively. There were 9 pulmonary sequestrations[all intralobar type] with the distribution of 5 in right lower lobe and 4 in left lower lobe. And associated anomalies were presented with arterial supply originating from thoracic aorta[8 cases], abdominal aorta[1 case] and with venous drainage into azygos vein[1 case]. They all were operated upon lower lobectomy [8 case], pneumonectomy[1 case] in case of pulmonary hypoplasia Congenital lobar emphysema and congenital cystic adenomatoid malformation had 4 cases respectively. Their affected lobes were as follows: the former were 3 in upper lobes, 1 in middle lobe and the latter were 3 in upper lobe, 1 in lower lobe. They were treated with lobectomy and segmentectomy. Diagnosis was aided by chest X - ray, bronchography, aortography, DSA and CT scan, They all were confirmed by pathologic exams. There were no hospital death but few minor morbidities such as, atelectasis-pneumonia[2], wound infection[2], prolonged chest tube placement[2]. We experienced surgical treatments of 47 cases for 10 years and reported them with literature review.

• Clinical and Experimental Pediatrics
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• v.61 no.1
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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.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.33 no.2
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• pp.176-179
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• 2011

Adenomatoid odontogenic tumors represent 3 to 7 percent of all odontogenic tumors. These tumors are more common in the maxilla than the mandible and usually include the anterior region. Clinically, the most common symptom is painless swelling and the tumor is associated with an unerupted tooth, typically a maxillary or mandibular cuspid. The adenomatoid odontogenic tumor appears radiographically as a unilocular radiolucency around the crown of an impacted tooth, resembling a dentigerous cyst. More often, it contains fine calcifications. Histopathologically, there is a thick wall cystic structure with a prominent intraluminal proliferation of the odontogenic epithelium. The most striking pattern is varying-sized solid nodules of spindle-shaped or cuboidal epithelial cells forming nests or rosette-like structures with minimal stromal connective tissues. Conspicuous within the cellular areas are structures of tubular or duct-like appearance. The duct-like spaces are lined with a single row of cuboidal or low columnar epithelial cells, of which the ovoid nuclei are polarized away from the luminal surface. Small foci of calcification may also be scattered throughout the tumor. These have been interpreted as abortive enamel formations. In some adenomatoid odontogenic tumors, the material has been interpreted as dentoid or cementum.

• 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.

• Imaging Science in Dentistry
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• v.39 no.2
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• pp.99-102
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• 2009

Adenomatoid odontogenic tumor (AOT) is a tumor of odontogenic epithelium with varying degrees of inductive changes in the connective tissue. The common radiographic appearance of AOT is a unilocular radiolucency associated with an unerupted tooth. Detectable radiopacities have been reported in many cases. We present a case of AOT in a 9-year old-female patient. Cystic lesion with numerous, punctuate radiopaque foci was observed on the anterior region of the mandible. These radiopacities were situated mostly on the buccal side of impacted tooth on the multi-planar images of cone beam computed tomograph. Characteristic duct like structures and amyloid like material were observed on histopathologic finding. (Korean J Oral Maxillofac Radiol2009; 39 : 99-102)