Pleuropulmonary Blastoma Developing in a Case of Misinterpreted Congenital Pulmonary Airway Malformation: a Case Report

Ghosh, Moupali; Islam, Nelofar; Ghosh, Arindam; Chaudhuri, Picklu; Saha, Koushik; Chatterjee, Uttara

doi:10.1080/15513815.2018.1520943

Cited by 9 publications

(11 citation statements)

References 24 publications

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“…Our paper describes a case of PPB, initially diagnosed as CPAM. After the establishment of International Pleuropulmonary Blastoma Registry, review of several cases has confirmed the possibility that lesions first diagnosed as CPAM were later diagnosed as PPB type I [ [1] , [2] , [3] , 6 , 9 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

Hypertensive pneumothorax with cystic lesions: Pleuropulmonary blastoma in an infant

Lamberti

Ghezzi

Riccaboni

et al. 2023

Respiratory Medicine Case Reports

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Section: Discussionmentioning

confidence: 99%

Hypertensive pneumothorax with cystic lesions: Pleuropulmonary blastoma in an infant

Lamberti

Ghezzi

Riccaboni

et al. 2023

Respiratory Medicine Case Reports

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“…However, even for asymptomatic patients, the possibility of repeated infection and malignant transformation are the reasons for pneumonectomy, which may outweigh the benefits of con servative management. CPAM can occasionally be a cystic mal formation, and it may be a pleuropulmonary blastoma induced by DICER1 mutation, albeit rarely (with an estimated incidence of 1 in 300,000) 20,25) . .…”

Section: Surgery and Complicationsmentioning

confidence: 99%

Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience

Lim¹,

Han²,

Shin³

et al. 2019

Neonatal Med

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Purpose: Congenital pulmonary airway malformation (CPAM)-a rare developmental anomaly-affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide com prehensive clinical data for the proper management of CPAM. Methods: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. Results: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive pre dictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent videoassisted thoracoscopy. Twentyfive and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. Conclusion: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with pre natally diagnosed CPAM and to guide appropriate timing of surgery.

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Section: Introductionmentioning

confidence: 99%

“…17 Many PPBs have a similar appearance to macrocystic CPAMs on computed tomography (CT) scans, the current criterion standard imaging modality to assess the lung parenchyma in children, based on several retrospective reviews. 15,[18][19][20][21][22][23] The reliability of CT in cases of PPB remains unknown. 24 Given ongoing concerns about misdiagnosing PPB as a benign macrocytic CPAM, we used a multi-institutional consortium registry to formally evaluate the CT characteristics of cystic PPB and benign CLMs.…”

Section: Introductionmentioning

confidence: 99%

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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Pleuropulmonary Blastoma Developing in a Case of Misinterpreted Congenital Pulmonary Airway Malformation: a Case Report

Cited by 9 publications

References 24 publications

Hypertensive pneumothorax with cystic lesions: Pleuropulmonary blastoma in an infant

Hypertensive pneumothorax with cystic lesions: Pleuropulmonary blastoma in an infant

Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience

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

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