Mucinous bronchioloalveolar carcinoma with K-ras mutation arising in type 1 congenital cystic adenomatoid malformation: a case report with review of the literature.
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Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare hamartomatous cystic lesion, characterized by the presence of large cysts, which are histopathologically lined by pseudostratified ciliated cells. It has been recognized that rare cases of type 1 CCAM show malignant transformation, usually bronchioloalveolar carcinoma (BAC) or adenocarcinoma. Herein, we describe a case of BAC arising in type 1 CCAM with K-ras mutation. A 9-year-old Japanese girl presented with fever. Computed tomography demonstrated large cystic lesions in her right lower lung. Histopathological study of the resected specimen revealed multiple cysts, which were lined by pseudostratified ciliated cells, and occasionally interspersed with mucous cells without atypia. A small focus of proliferation of columnar cells showing lepidic growth pattern was present. These columnar cells had abundant mucin in the cytoplasm and mildly to moderately enlarged nuclei. Accordingly, a diagnosis of BAC arising in type 1 CCAM was made. Polymerase chain reaction analysis revealed K-ras mutation at codon 12 in the BAC component. The presence of mucous cell/goblet cell hyperplasia and atypical adenomatous hyperplasia is a well known phenomenon in type 1 CCAM. A recent study clearly demonstrated K-ras mutation in these lesions, which are thought to be precursors of BAC. Therefore, the concept of malignant transformation in the sequence from type 1 CCAM to mucous cell hyperplasia to atypical adenomatous hyperplasia to BAC and invasive adenocarcinoma due to K-ras mutation has been proposed. Careful histopathological analysis is important for evaluation of malignant lesions in type 1 CCAM. (+info)
EGFR mutation of adenocarcinoma in congenital cystic adenomatoid malformation/congenital pulmonary airway malformation: a case report.
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Prenatal diagnosis of cystic adenomatoid malformation of the lung.
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Cystic adenomatoid malformation of the lung (CAM) is an uncommon malformation in which normal tissue is replaced by cysts of various sizes. This malformation may be diagnosed prenatally with ultrasound by visualization of cystic masses in the fetal chest, which may occur in association with fetal hydrops. Three cases of fetal hydrops secondary to cystic adenomatoid malformation of the lung are described; in one case cystic lesions in the chest were clearly demonstrated. (+info)
The vanishing fetal intrathoracic mass: consider an obstructing mucous plug.
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An intrathoracic homogeneous hyperechogenic mass diagnosed in the second trimester of pregnancy represents a challenge to the sonographer. A disappearance of such a finding is an enigma. We have recently encountered such a case in which a mucous plug obstructing a main bronchus was the causative factor. To the best of our knowledge, this is the first report of such an etiology in cases of vanishing fetal intrathoracic masses diagnosed by ultrasonography. (+info)
Adult presentation of congenital cystic adenomatoid malformation of the lung: a case report.
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Adult presentation of congenital cystic adenomatoid malformation(CCAM) of the lung is so rare that only 5 cases have been reported in the literature to date. We report the case of a 19-year-old female with CCAM in the left lower lobe. Computed tomography showed a multilobulated cystic lesion with multiple air-fluid levels and also showed focal enhancement of the solid component in the eccentrical portion of the lesion. Thoracic aortogram and selected bronchial arteriograms show a slightly enlarged and tortuous bronchial artery feeding the multilobulated cystic lesion. We present the clinical and radiological features of our case with a brief review of the literature. (+info)
Fetal lung masses: prenatal course and outcome.
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We describe 25 cases of echogenic or complex fetal lung masses seen sonographically and suspected of being cystic adenomatoid malformations or sequestrations of the lung. On the basis of prenatal sonographic appearance, 40% of fetuses were suspected of having type 1, 20% type 2, and 40% type 3 cystic adenomatoid malformations or sequestrations. Sixteen (64%) of the 25 fetuses with lung masses survived the perinatal period, and 80% of the nonaborted fetuses survived. Eleven infants (69% of liveborns) had no respiratory symptoms at birth. Nine of the survivors underwent surgical resections of their masses after birth, whereas the other seven children are being followed conservatively. The postnatal diagnoses revealed that four of the fetuses had sequestrations, one had a sequestration with elements consistent with cystic adenomatoid malformation, and two who were thought to have type 1 cystic adenomatoid malformation had an esophageal duplication cyst and a thoracic neuroblastoma, respectively. All the other infants who had a pathologic diagnosis or postnatal imaging had cystic adenomatoid malformations. Increasing mediastinal shift was associated with decreasing survival as 90% of fetuses with no mediastinal shift are alive, whereas 50% of the nonaborted fetuses with a severe mediastinal shift survived. Follow-up scans in utero were available in 15 cases. The size of the mass became smaller in 53% during gestation. Seventy-one percent of pregnancies had normal amniotic fluid volumes and 29% were complicated by polyhydramnios. Survival of nonaborted fetuses was 100% in pregnancies with normal amniotic fluid compared with 50% in those with polyhydramnios. Eight percent of the fetuses with chest masses had additional structural abnormalities and were karyotypically abnormal. In conclusion, many fetuses with lung masses show improvement of the sonographic findings in utero, and many infants may not be symptomatic at birth. Of the survivors in this series, only slightly greater than half underwent surgery. (+info)
Clinical, radiographic and lung function features of diffuse congenital cystic adenomatoid malformation of the lung in an adult.
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Congenital cystic adenomatoid malformation of the bronchi with bilateral lung involvement has only previously been reported in one possible adult case. This report describes a young man with bilateral diffuse lung involvement, characteristic histological features, and a mixed restrictive/obstructive lung function pattern. (+info)
Sonographic detection of bilateral fetal chest masses: report of three cases.
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Fetal chest masses are rare lesions that can be detected on prenatal sonography. This report of three cases of bilateral fetal chest masses (two bilateral congenital cystic adenomatoid malformations of the lung and one case of bilateral pulmonary sequestrations) serves to emphasize the occasional occurrence of bilateral masses and the variability in prognoses. (+info)