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Pediatrics
Diagnosis
Genitourinary
Renal Masses
Multilocular Cystic Nephroma
Multilocular Cystic Nephroma
Alexander J. Towbin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Multilocular cystic nephroma (MLCN): Benign mixed mesenchymal & epithelial renal neoplasm

            IMAGING

            • General Features

              • Radiographic Findings

                • CT/MR/US

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
                                        2. Stanescu AL et al: Pediatric renal neoplasms: MR imaging-based practical diagnostic approach. Magn Reson Imaging Clin N Am. 27(2):279-90, 2019
                                        3. Kurian JJ et al: Multiloculated cystic renal tumors of childhood: has the final word been spoken. J Indian Assoc Pediatr Surg. 23(1):22-6, 2018
                                        4. Chung EM et al: Renal tumors of childhood: radiologic-pathologic correlation part 1. The 1st decade: from the radiologic pathology archives. Radiographics. 36(2):499-522, 2016
                                        5. 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
                                        6. Wu MK et al: Evolution of renal cysts to anaplastic sarcoma of kidney in a child with DICER1 syndrome. Pediatr Blood Cancer. 63(7):1272-5, 2016
                                        7. Granja MF et al: Multilocular cystic nephroma: a systematic literature review of the radiologic and clinical rindings. AJR Am J Roentgenol. 205(6):1188-93, 2015
                                        8. Doros LA et al: DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma. Mod Pathol. 27(9):1267-80, 2014
                                        9. Silver IM et al: Best cases from the AFIP: multilocular cystic renal tumor: cystic nephroma. Radiographics. 28(4):1221-5; discussion 1225-6, 2008
                                        10. Hopkins JK et al: Best cases from the AFIP: cystic nephroma. Radiographics. 24(2):589-93, 2004
                                        11. Agrons GA et al: Multilocular cystic renal tumor in children: radiologic-pathologic correlation. Radiographics. 15(3):653-69, 1995
                                        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

                                                  TERMINOLOGY

                                                  • Definitions

                                                    • Multilocular cystic nephroma (MLCN): Benign mixed mesenchymal & epithelial renal neoplasm

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT/MR/US

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
                                                                              2. Stanescu AL et al: Pediatric renal neoplasms: MR imaging-based practical diagnostic approach. Magn Reson Imaging Clin N Am. 27(2):279-90, 2019
                                                                              3. Kurian JJ et al: Multiloculated cystic renal tumors of childhood: has the final word been spoken. J Indian Assoc Pediatr Surg. 23(1):22-6, 2018
                                                                              4. Chung EM et al: Renal tumors of childhood: radiologic-pathologic correlation part 1. The 1st decade: from the radiologic pathology archives. Radiographics. 36(2):499-522, 2016
                                                                              5. 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
                                                                              6. Wu MK et al: Evolution of renal cysts to anaplastic sarcoma of kidney in a child with DICER1 syndrome. Pediatr Blood Cancer. 63(7):1272-5, 2016
                                                                              7. Granja MF et al: Multilocular cystic nephroma: a systematic literature review of the radiologic and clinical rindings. AJR Am J Roentgenol. 205(6):1188-93, 2015
                                                                              8. Doros LA et al: DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma. Mod Pathol. 27(9):1267-80, 2014
                                                                              9. Silver IM et al: Best cases from the AFIP: multilocular cystic renal tumor: cystic nephroma. Radiographics. 28(4):1221-5; discussion 1225-6, 2008
                                                                              10. Hopkins JK et al: Best cases from the AFIP: cystic nephroma. Radiographics. 24(2):589-93, 2004
                                                                              11. Agrons GA et al: Multilocular cystic renal tumor in children: radiologic-pathologic correlation. Radiographics. 15(3):653-69, 1995