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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, multilocular cystic nephroma (MLCN), ovarian sex cord-stromal tumors (especially Sertoli-Leydig cell tumor), multinodular goiter, & differentiated thyroid carcinoma
- DICER1 mutation
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
- Coleman A et al: Pleuropulmonary blastoma in a neonate diagnosed prenatally as congenital pulmonary airway malformation. Fetal Diagn Ther. 39(3):234-7, 2016
- Faure A et al: DICER1 pleuropulmonary blastoma familial tumour predisposition syndrome: What the paediatric urologist needs to know. J Pediatr Urol. 12(1):5-10, 2016
- Feinberg A et al: Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features? J Pediatr Surg. 51(1):33-7, 2016
- Messinger YH et al: Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the International Pleuropulmonary Blastoma Registry. Cancer. 121(2):276-85, 2015
- Sabapathy DG et al: Radiographic screening of infants and young children with genetic predisposition for rare malignancies: DICER1 mutations and pleuropulmonary blastoma. AJR Am J Roentgenol. 204(4):W475-82, 2015
- Schultz KA et al: Judicious DICER1 testing and surveillance imaging facilitates early diagnosis and cure of pleuropulmonary blastoma. Pediatr Blood Cancer. 61(9):1695-7, 2014
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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, multilocular cystic nephroma (MLCN), ovarian sex cord-stromal tumors (especially Sertoli-Leydig cell tumor), multinodular goiter, & differentiated thyroid carcinoma
- DICER1 mutation
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
- Coleman A et al: Pleuropulmonary blastoma in a neonate diagnosed prenatally as congenital pulmonary airway malformation. Fetal Diagn Ther. 39(3):234-7, 2016
- Faure A et al: DICER1 pleuropulmonary blastoma familial tumour predisposition syndrome: What the paediatric urologist needs to know. J Pediatr Urol. 12(1):5-10, 2016
- Feinberg A et al: Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features? J Pediatr Surg. 51(1):33-7, 2016
- Messinger YH et al: Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the International Pleuropulmonary Blastoma Registry. Cancer. 121(2):276-85, 2015
- Sabapathy DG et al: Radiographic screening of infants and young children with genetic predisposition for rare malignancies: DICER1 mutations and pleuropulmonary blastoma. AJR Am J Roentgenol. 204(4):W475-82, 2015
- Schultz KA et al: Judicious DICER1 testing and surveillance imaging facilitates early diagnosis and cure of pleuropulmonary blastoma. Pediatr Blood Cancer. 61(9):1695-7, 2014
