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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, 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

                                              1. Kunisaki SM et al: Pleuropulmonary blastoma in pediatric lung lesions. Pediatrics. 147(4), 2021
                                              2. Madaan PK et al: Pleuropulmonary blastoma: a report of three cases and review of literature. Radiol Case Rep. 16(10):2862-8, 2021
                                              3. 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
                                              4. Dehner LP et al: Pleuropulmonary blastoma: more than a lung neoplasm of childhood. Mo Med. 116(3):206-10, 2019
                                              5. Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
                                              6. Lichtenberger JP 3rd et al: Primary lung tumors in children: radiologic-pathologic correlation from the radiologic pathology archives. Radiographics. 38(7):2151-72, 2018
                                              7. 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
                                              8. Bueno MT et al: Pediatric imaging in DICER1 syndrome. Pediatr Radiol. 47(10):1292-301, 2017
                                              9. 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

                                                                                          1. Kunisaki SM et al: Pleuropulmonary blastoma in pediatric lung lesions. Pediatrics. 147(4), 2021
                                                                                          2. Madaan PK et al: Pleuropulmonary blastoma: a report of three cases and review of literature. Radiol Case Rep. 16(10):2862-8, 2021
                                                                                          3. 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
                                                                                          4. Dehner LP et al: Pleuropulmonary blastoma: more than a lung neoplasm of childhood. Mo Med. 116(3):206-10, 2019
                                                                                          5. Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
                                                                                          6. Lichtenberger JP 3rd et al: Primary lung tumors in children: radiologic-pathologic correlation from the radiologic pathology archives. Radiographics. 38(7):2151-72, 2018
                                                                                          7. 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
                                                                                          8. Bueno MT et al: Pediatric imaging in DICER1 syndrome. Pediatr Radiol. 47(10):1292-301, 2017
                                                                                          9. 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