Rare malignant embryonal mesenchymal neoplasm of lung & pleura that arises during organ development
Associated Syndromes
DICER1 mutation
Associated with PPB, cystic nephroma (CN), ovarian sex cord-stromal tumors (especially Sertoli-Leydig cell tumor), multinodular goiter, differentiated thyroid carcinoma, pineoblastoma, & ovarian or cervical rhabdomyosarcoma
IMAGING
General Features
Radiographic Findings
CT Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Kunisaki SM et al: Pleuropulmonary blastoma in pediatric lung lesions. Pediatrics. 147(4), 2021
Madaan PK et al: Pleuropulmonary blastoma: a report of three cases and review of literature. Radiol Case Rep. 16(10):2862-8, 2021
Adams NC et al: Fetal ultrasound and magnetic resonance imaging: a primer on how to interpret prenatal lung lesions. Pediatr Radiol. 50(13):1839-54, 2020
Dehner LP et al: Pleuropulmonary blastoma: more than a lung neoplasm of childhood. Mo Med. 116(3):206-10, 2019
Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
Lichtenberger JP 3rd et al: Primary lung tumors in children: radiologic-pathologic correlation from the radiologic pathology archives. Radiographics. 38(7):2151-72, 2018
van Engelen K et al: DICER1 syndrome: approach to testing and management at a large pediatric tertiary care center. Pediatr Blood Cancer. 65(1), 2018
Bueno MT et al: Pediatric imaging in DICER1 syndrome. Pediatr Radiol. 47(10):1292-301, 2017
Waelti SL et al: Neonatal congenital lung tumors - the importance of mid-second-trimester ultrasound as a diagnostic clue. Pediatr Radiol. 47(13):1766-75, 2017
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Pleuropulmonary blastoma (PPB)
Definitions
Rare malignant embryonal mesenchymal neoplasm of lung & pleura that arises during organ development
Associated Syndromes
DICER1 mutation
Associated with PPB, cystic nephroma (CN), ovarian sex cord-stromal tumors (especially Sertoli-Leydig cell tumor), multinodular goiter, differentiated thyroid carcinoma, pineoblastoma, & ovarian or cervical rhabdomyosarcoma
IMAGING
General Features
Radiographic Findings
CT Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Kunisaki SM et al: Pleuropulmonary blastoma in pediatric lung lesions. Pediatrics. 147(4), 2021
Madaan PK et al: Pleuropulmonary blastoma: a report of three cases and review of literature. Radiol Case Rep. 16(10):2862-8, 2021
Adams NC et al: Fetal ultrasound and magnetic resonance imaging: a primer on how to interpret prenatal lung lesions. Pediatr Radiol. 50(13):1839-54, 2020
Dehner LP et al: Pleuropulmonary blastoma: more than a lung neoplasm of childhood. Mo Med. 116(3):206-10, 2019
Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
Lichtenberger JP 3rd et al: Primary lung tumors in children: radiologic-pathologic correlation from the radiologic pathology archives. Radiographics. 38(7):2151-72, 2018
van Engelen K et al: DICER1 syndrome: approach to testing and management at a large pediatric tertiary care center. Pediatr Blood Cancer. 65(1), 2018
Bueno MT et al: Pediatric imaging in DICER1 syndrome. Pediatr Radiol. 47(10):1292-301, 2017
Waelti SL et al: Neonatal congenital lung tumors - the importance of mid-second-trimester ultrasound as a diagnostic clue. Pediatr Radiol. 47(13):1766-75, 2017
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