Pleuropulmonary Blastoma in a Neonate Diagnosed Prenatally as Congenital Pulmonary Airway Malformation

Coleman, Alan; Kline‐Fath, Beth M.; Stanek, Jerzy; Lim, Foong‐Yen

doi:10.1159/000365352

Cited by 14 publications

(11 citation statements)

References 5 publications

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“…Fetal congenital lung lesions (CLLs) are a heterogeneous group of lesions that most commonly include entities such as congenital pulmonary airway malformation (CPAM) and bronchopulmonary sequestration (BPS), less frequent entities such as congenital lobar emphysema and bronchial atresia, and some rare forms of embryonal tumors, including pleuropulmonary blastoma …”

mentioning

confidence: 99%

Utility of Fetal Cardiac Axis and Cardiac Position Assessment in Predicting Neonatal Respiratory Morbidity in Fetal Congenital Lung Lesions

Tuzović

Copel

Stitelman

et al. 2019

J of Ultrasound Medicine

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Objectives-To assess the diagnostic performance of the fetal cardiac axis (CA) and/or cardiac position (CP) versus the congenital pulmonary malformation volume ratio (CVR) in predicting any and severe neonatal respiratory morbidity in fetal congenital lung lesions.Methods-This work was an 11-year retrospective cohort study. The sensitivity, specificity, positive predictive value, and negative predictive value of CA and/or CP assessment in prediction of respiratory morbidity were calculated before 24 weeks' gestation and between 24 and 32 weeks and compared to CVR cutoffs obtained from the literature.Results-Fifty-three patients were included. CA and/or CP abnormalities were present in 45% and 38% of patients before 24 weeks and between 24 and 32 weeks and were significantly more common in left-versus rightsided lesions (60% versus 17%; P = .003). The sensitivity, specificity, positive predictive value, and negative predictive value of an abnormal CA and/or CP for any and severe respiratory morbidity were 0.67, 0.61, 0.33, and 0.86 and 0.8, 0.58, 0.17, and 0.97 before 24 weeks and 0.75, 0.73, 0.45, and 0.91 and 0.8, 0.67, 0.20, and 0.97 between 24 and 32 weeks, respectively. An abnormal CA and/or CP had higher sensitivity for any respiratory morbidity compared to the CVR at 0.5 and 0.8 cutoffs both before 24 weeks and between 24 and 32 weeks (P < .05).Conclusions-An abnormal CA and/or CP before 24 weeks and between 24 and 32 weeks has higher sensitivity for the detection of any respiratory morbidity at birth compared to the CVR at both 0.5 and 0.8 cutoffs. A normal CA and CP have a high negative predictive value for excluding any respiratory morbidity at birth both before 24 weeks and between 24 and 32 weeks.

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mentioning

confidence: 99%

Utility of Fetal Cardiac Axis and Cardiac Position Assessment in Predicting Neonatal Respiratory Morbidity in Fetal Congenital Lung Lesions

Tuzović

Copel

Stitelman

et al. 2019

J of Ultrasound Medicine

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“… 21 , 38 , 39 Less than 5% of PPBs are detected prenatally. 18 , 20 , 40 , 41 The only radiologic feature that is thought to be pathognomic for benign disease is the presence of a systemic feeding vessel, 16 , 18 and our study radiologists correctly excluded malignancy in their review of all confirmed BPS lesions. The significance of other image-based characteristics in identifying PPB is more controversial.…”

Section: Discussionmentioning

confidence: 66%

Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children

et al. 2022

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IMPORTANCEThe ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear.OBJECTIVE To 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 MEASURES Accuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors. RESULTS Among 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.

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“…It does not appear that there is a relevant correlation between prenatal sonographic appearance of these lesions and the final histopathology diagnosis, which is illustrated well in the report by Coleman et al [1,2] . PPB is a rare embryonic tumor which should be included in the differential diagnosis of any fetal congenital lung lesion diagnosed prenatally.…”

Section: Disclosure Statementmentioning

confidence: 86%

“…The lesion was removed in the early neonatal period due to respiratory morbidity [1] . This report illustrates several important points pertinent to the management of prenatally diagnosed fetal congenital lung lesions.…”

Section: Disclosure Statementmentioning

confidence: 99%

Fetal Congenital Lung Lesions, DICER1 Syndrome, and Family History as a Risk Assessment Tool

Tuzović

2016

Fetal Diagn Ther

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Pleuropulmonary Blastoma in a Neonate Diagnosed Prenatally as Congenital Pulmonary Airway Malformation

Cited by 14 publications

References 5 publications

Utility of Fetal Cardiac Axis and Cardiac Position Assessment in Predicting Neonatal Respiratory Morbidity in Fetal Congenital Lung Lesions

Utility of Fetal Cardiac Axis and Cardiac Position Assessment in Predicting Neonatal Respiratory Morbidity in Fetal Congenital Lung Lesions

Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children

Fetal Congenital Lung Lesions, DICER1 Syndrome, and Family History as a Risk Assessment Tool

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