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Nephroblastomatosis
Alexander J. Towbin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Nephrogenic rests (NR): Metanephric blastema that persists after birth; precursor to Wilms tumor
            • Nephroblastomatosis: Presence of multiple or diffuse NR
            • Intralobar NR: Occur anywhere in renal parenchyma
            • Perilobar NR: Occur in peripheral cortex
          • Associated Syndromes

            • Perilobar NR associated with Beckwith-Wiedemann syndrome, hemihypertrophy, Perlman syndrome, trisomy 18
            • Intralobar NR associated with Drash syndrome, sporadic aniridia, WAGR syndrome

          IMAGING

          • General Features

            • Ultrasonographic Findings

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      • Differentiation From Wilms tumor

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Littooij AS et al: Intra- and interobserver variability of whole-tumour apparent diffusion coefficient measurements in nephroblastoma: a pilot study. Pediatr Radiol. 45(11):1651-60, 2015
                                            2. Uslu L et al: Value of 18F-FDG PET and PET/CT for evaluation of pediatric malignancies. J Nucl Med. 56(2):274-86, 2015
                                            3. Cox SG et al: Magnetic resonance imaging versus histopathology in Wilms tumor and nephroblastomatosis: 3 examples of noncorrelation. J Pediatr Hematol Oncol. 36(2):e81-4, 2014
                                            4. Stabouli S et al: Perilobar nephroblastomatosis: natural history and management. Case Rep Pediatr. 2014:756819, 2014
                                            5. Sethi AT et al: Best cases from the AFIP: Wilms tumor in the setting of bilateral nephroblastomatosis. Radiographics. 30(5):1421-5, 2010
                                            6. Perlman EJ et al: Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients. Pediatr Blood Cancer. 46(2):203-21, 2006
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Definitions

                                                      • Nephrogenic rests (NR): Metanephric blastema that persists after birth; precursor to Wilms tumor
                                                      • Nephroblastomatosis: Presence of multiple or diffuse NR
                                                      • Intralobar NR: Occur anywhere in renal parenchyma
                                                      • Perilobar NR: Occur in peripheral cortex
                                                    • Associated Syndromes

                                                      • Perilobar NR associated with Beckwith-Wiedemann syndrome, hemihypertrophy, Perlman syndrome, trisomy 18
                                                      • Intralobar NR associated with Drash syndrome, sporadic aniridia, WAGR syndrome

                                                    IMAGING

                                                    • General Features

                                                      • Ultrasonographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Nuclear Medicine Findings

                                                              • Imaging Recommendations

                                                                • Differentiation From Wilms tumor

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Littooij AS et al: Intra- and interobserver variability of whole-tumour apparent diffusion coefficient measurements in nephroblastoma: a pilot study. Pediatr Radiol. 45(11):1651-60, 2015
                                                                                      2. Uslu L et al: Value of 18F-FDG PET and PET/CT for evaluation of pediatric malignancies. J Nucl Med. 56(2):274-86, 2015
                                                                                      3. Cox SG et al: Magnetic resonance imaging versus histopathology in Wilms tumor and nephroblastomatosis: 3 examples of noncorrelation. J Pediatr Hematol Oncol. 36(2):e81-4, 2014
                                                                                      4. Stabouli S et al: Perilobar nephroblastomatosis: natural history and management. Case Rep Pediatr. 2014:756819, 2014
                                                                                      5. Sethi AT et al: Best cases from the AFIP: Wilms tumor in the setting of bilateral nephroblastomatosis. Radiographics. 30(5):1421-5, 2010
                                                                                      6. Perlman EJ et al: Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients. Pediatr Blood Cancer. 46(2):203-21, 2006