Dataset Information

Congenital cystic adenomatoid malformations of the lung: an epithelial transcriptomic approach.

ABSTRACT: BACKGROUND:The pathophysiology of congenital cystic adenomatoid malformations (CCAM) of the lung remains poorly understood. AIM:This study aimed to identify more precisely the molecular mechanisms limited to a compartment of lung tissue, through a transcriptomic analysis of the epithelium of macrocystic forms. METHODS:Tissue fragments displaying CCAM were obtained during planned surgical resections. Epithelial mRNA was obtained from cystic and normal areas after laser capture microdissection (LCM). Transcriptomic analyses were performed and the results were confirmed by RT-PCR and immunohistochemistry in independent samples. RESULTS:After controlling for RNA quality, we analysed the transcriptomes of six cystic areas and five control areas. In total, 393 transcripts were differentially expressed in the epithelium, between CCAM and control areas. The most highly redundant genes involved in biological functions and signalling pathways differentially expressed between CCAM and control epithelium included TGFB2, TGFBR1, and MAP 2?K1. These genes were considered particularly relevant as they have been implicated in branching morphogenesis. RT-qPCR analysis confirmed in independent samples that TGFBR1 was more strongly expressed in CCAM than in control tissues (p?

SUBMITTER: Lezmi G

PROVIDER: S-EPMC7001206 | biostudies-literature | 2020 Feb

REPOSITORIES: biostudies-literature

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Congenital cystic adenomatoid malformations of the lung: an epithelial transcriptomic approach.

Lezmi Guillaume G Vibhushan Shamila S Bevilaqua Claudia C Crapart Nicolas N Cagnard Nicolas N Khen-Dunlop Naziha N Boyle-Freyssaut Christine C Hadchouel Alice A Delacourt Christophe C

Respiratory research 20200204 1

<h4>Background</h4>The pathophysiology of congenital cystic adenomatoid malformations (CCAM) of the lung remains poorly understood.<h4>Aim</h4>This study aimed to identify more precisely the molecular mechanisms limited to a compartment of lung tissue, through a transcriptomic analysis of the epithelium of macrocystic forms.<h4>Methods</h4>Tissue fragments displaying CCAM were obtained during planned surgical resections. Epithelial mRNA was obtained from cystic and normal areas after laser captu ...[more]

PMID: 32019538

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Project description:ImportanceThe ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear.ObjectiveTo assess whether chest CT can detect malignant tumors among postnatally detected lung lesions in children.Design, setting, and participantsThis retrospective multicenter case-control study used a consortium database of 521 pathologically confirmed primary lung lesions from January 1, 2009, through December 31, 2015, to assess diagnostic accuracy. Preoperative CT scans of children with cystic PPB (cases) were selected and age-matched with CT scans from patients with postnatally detected congenital lung malformations (controls). Statistical analysis was performed from January 18 to September 6, 2020. Preoperative CT scans were interpreted independently by 9 experienced pediatric radiologists in a blinded fashion and analyzed from January 24, 2019, to September 6, 2020.Main outcomes and measuresAccuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors.ResultsAmong 477 CT scans identified (282 boys [59%]; median age at CT, 3.6 months [IQR, 1.2-7.2 months]; median age at resection, 6.9 months [IQR, 4.2-12.8 months]), 40 cases were extensively reviewed; 9 cases (23%) had pathologically confirmed cystic PPB. The median age at CT was 7.3 months (IQR, 2.9-22.4 months), and median age at resection was 8.7 months (IQR, 5.0-24.4 months). The sensitivity of CT for detecting PPB was 58%, and the specificity was 83%. High suspicion for malignancy correlated with PPB pathology (odds ratio, 13.5; 95% CI, 2.7-67.3; P = .002). There was poor interrater reliability (κ = 0.36 [range, 0.06-0.64]; P < .001) and no significant difference in specific imaging characteristics between PPB and benign cystic lesions. The overall accuracy rate for distinguishing benign vs malignant lesions was 81%.Conclusions and relevanceThis study suggests that chest CT, the current criterion standard imaging modality to assess the lung parenchyma, may not accurately and reliably distinguish PPB from benign congenital lung malformations in children. In any cystic lung lesion without a prenatal diagnosis, operative management to confirm pathologic diagnosis is warranted.

Dataset Information

Congenital cystic adenomatoid malformations of the lung: an epithelial transcriptomic approach.

Publications

Congenital cystic adenomatoid malformations of the lung: an epithelial transcriptomic approach.

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