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Renal Rhabdoid Tumor
Ethan A. Smith, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Malignant rhabdoid tumor, extracranial rhabdoid tumor
            • Definitions

              • Rare, highly aggressive neoplasm of young children with poor prognosis
              • Most commonly occurs in kidney

            IMAGING

            • General Features

              • Radiographic Findings

                • Ultrasonographic Findings

                  • CT Findings

                    • MR Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Nemes K et al: Clinical and genetic risk factors define two risk groups of extracranial malignant rhabdoid tumours (eMRT/RTK). Eur J Cancer. 142:112-22, 2021
                                        2. Treece AL: Pediatric renal tumors: updates in the molecular era. Surg Pathol Clin. 13(4):695-718, 2020
                                        3. Stanescu AL et al: Pediatric renal neoplasms: MR imaging-based practical diagnostic approach. Magn Reson Imaging Clin N Am. 27(2):279-90, 2019
                                        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. Farmakis SG et al: Rhabdoid tumor: an aggressive renal medullary tumor of childhood. J Comput Assist Tomogr. 39(1):44-6, 2015
                                        6. Hong CR et al: Extra-cranial malignant rhabdoid tumor in children: a single institute experience. Cancer Res Treat. 47(4):889-96, 2015
                                        7. Gee MS et al: Magnetic resonance imaging of the pediatric kidney: benign and malignant masses. Magn Reson Imaging Clin N Am. 21(4):697-715, 2013
                                        8. Royer-Pokora B: Genetics of pediatric renal tumors. Pediatr Nephrol. 28(1):13-23, 2013
                                        9. Geller E et al: Renal neoplasms of childhood. Radiol Clin North Am. 49(4):689-709, vi, 2011
                                        10. van den Heuvel-Eibrink MM et al: Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group. Pediatr Blood Cancer. 56(5):733-7, 2011
                                        11. Morgenstern DA et al: Clinical and pathological features of paediatric malignant rhabdoid tumours. Pediatr Blood Cancer. 54(1):29-34, 2010
                                        12. Tomlinson GE et al: Rhabdoid tumor of the kidney in the National Wilms' Tumor Study: age at diagnosis as a prognostic factor. J Clin Oncol. 23(30):7641-5, 2005
                                        13. Agrons GA et al: Rhabdoid tumor of the kidney in children: a comparative study of 21 cases. AJR Am J Roentgenol. 168(2):447-51, 1997
                                        14. Nemes K et al: Rhabdoid tumor predisposition syndrome, 1993
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Pathology

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Malignant rhabdoid tumor, extracranial rhabdoid tumor
                                                  • Definitions

                                                    • Rare, highly aggressive neoplasm of young children with poor prognosis
                                                    • Most commonly occurs in kidney

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • Ultrasonographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Nemes K et al: Clinical and genetic risk factors define two risk groups of extracranial malignant rhabdoid tumours (eMRT/RTK). Eur J Cancer. 142:112-22, 2021
                                                                              2. Treece AL: Pediatric renal tumors: updates in the molecular era. Surg Pathol Clin. 13(4):695-718, 2020
                                                                              3. Stanescu AL et al: Pediatric renal neoplasms: MR imaging-based practical diagnostic approach. Magn Reson Imaging Clin N Am. 27(2):279-90, 2019
                                                                              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. Farmakis SG et al: Rhabdoid tumor: an aggressive renal medullary tumor of childhood. J Comput Assist Tomogr. 39(1):44-6, 2015
                                                                              6. Hong CR et al: Extra-cranial malignant rhabdoid tumor in children: a single institute experience. Cancer Res Treat. 47(4):889-96, 2015
                                                                              7. Gee MS et al: Magnetic resonance imaging of the pediatric kidney: benign and malignant masses. Magn Reson Imaging Clin N Am. 21(4):697-715, 2013
                                                                              8. Royer-Pokora B: Genetics of pediatric renal tumors. Pediatr Nephrol. 28(1):13-23, 2013
                                                                              9. Geller E et al: Renal neoplasms of childhood. Radiol Clin North Am. 49(4):689-709, vi, 2011
                                                                              10. van den Heuvel-Eibrink MM et al: Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group. Pediatr Blood Cancer. 56(5):733-7, 2011
                                                                              11. Morgenstern DA et al: Clinical and pathological features of paediatric malignant rhabdoid tumours. Pediatr Blood Cancer. 54(1):29-34, 2010
                                                                              12. Tomlinson GE et al: Rhabdoid tumor of the kidney in the National Wilms' Tumor Study: age at diagnosis as a prognostic factor. J Clin Oncol. 23(30):7641-5, 2005
                                                                              13. Agrons GA et al: Rhabdoid tumor of the kidney in children: a comparative study of 21 cases. AJR Am J Roentgenol. 168(2):447-51, 1997
                                                                              14. Nemes K et al: Rhabdoid tumor predisposition syndrome, 1993