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Pleuropulmonary Blastoma
Alexander J. Towbin, MD
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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

              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

                                              1. Coleman A et al: Pleuropulmonary blastoma in a neonate diagnosed prenatally as congenital pulmonary airway malformation. Fetal Diagn Ther. 39(3):234-7, 2016
                                              2. 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
                                              3. 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
                                              4. 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
                                              5. 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
                                              6. 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
                                              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, multilocular cystic nephroma (MLCN), ovarian sex cord-stromal tumors (especially Sertoli-Leydig cell tumor), multinodular goiter, & differentiated thyroid carcinoma

                                                          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

                                                                                          1. Coleman A et al: Pleuropulmonary blastoma in a neonate diagnosed prenatally as congenital pulmonary airway malformation. Fetal Diagn Ther. 39(3):234-7, 2016
                                                                                          2. 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
                                                                                          3. 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
                                                                                          4. 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
                                                                                          5. 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
                                                                                          6. 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