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Pediatrics
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Brain
Cysts and Neoplasms
Atypical Teratoid/Rhabdoid Tumor
Atypical Teratoid/Rhabdoid Tumor
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Malignant rhabdoid tumor of brain
                • Intracranial rhabdoid tumor

              • Definitions

                • Atypical teratoid/rhabdoid tumor (AT/RT): Malignant embryonal tumor of CNS in early childhood
                  • Closely related to renal & soft tissue rhabdoid tumors

              IMAGING

              • General Features

                • CT Findings

                  • MR 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. Zhang M et al: Radiomic phenotypes distinguish atypical teratoid/rhabdoid tumors from medulloblastoma. AJNR Am J Neuroradiol. 42(9):1702-8, 2021
                                            2. Tamrazi B et al: Pediatric atypical teratoid/rhabdoid tumors of the brain: identification of metabolic subgroups using in vivo 1H-MR spectroscopy. AJNR Am J Neuroradiol. 40(5):872-7, 2019
                                            3. Nowak J et al: Magnetic resonance imaging surrogates of molecular subgroups in atypical teratoid/rhabdoid tumor. Neuro Oncol. 20(12):1672-9, 2018
                                            4. Fischer-Valuck BW et al: Assessment of the treatment approach and survival outcomes in a modern cohort of patients with atypical teratoid rhabdoid tumors using the National Cancer Database. Cancer. 123(4):682-7, 2017
                                            5. Katz JS et al: Cerebellopontine angle tumors in young children, displaying cranial nerve deficits, and restricted diffusion on diffusion-weighted imaging: a new clinical triad for atypical teratoid/rhabdoid tumors. Childs Nerv Syst. 33(5):833-8, 2017
                                            6. Frühwald MC et al: Atypical teratoid/rhabdoid tumors-current concepts, advances in biology, and potential future therapies. Neuro Oncol. 18(6):764-78, 2016
                                            7. Johann PD et al: Atypical teratoid/rhabdoid tumors are comprised of three epigenetic subgroups with distinct enhancer landscapes. Cancer Cell. 29(3):379-93, 2016
                                            8. DiPatri AJ Jr et al: Atypical teratoid rhabdoid tumors of the posterior fossa in children. Childs Nerv Syst. 31(10):1717-28, 2015
                                            9. Ho DM et al: Integrated genomics has identified a new AT/RT-like yet INI1-positive brain tumor subtype among primary pediatric embryonal tumors. BMC Med Genomics. 8:32, 2015
                                            10. Lau CS et al: Atypical teratoid rhabdoid tumors: a population-based clinical outcomes study involving 174 patients from the Surveillance, Epidemiology, and End Results database (1973-2010). Cancer Manag Res. 7:301-9, 2015
                                            11. Torchia J et al: Molecular subgroups of atypical teratoid rhabdoid tumours in children: an integrated genomic and clinicopathological analysis. Lancet Oncol. 16(5):569-82, 2015
                                            12. Ostrom QT et al: CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2007-2011. Neuro Oncol. 16 Suppl 4:iv1-63, 2014
                                            13. Slavc I et al: Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012. Cancer Med. 3(1):91-100, 2014
                                            14. Au Yong KJ et al: How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors? Pediatr Radiol. 43(3):347-54, 2013
                                            15. Bruggers CS et al: Magnetic resonance imaging spectroscopy in pediatric atypical teratoid rhabdoid tumors of the brain. J Pediatr Hematol Oncol. 36(6):341-5, 2013
                                            16. Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
                                            17. Koral K et al: Imaging characteristics of atypical teratoid-rhabdoid tumor in children compared with medulloblastoma. AJR Am J Roentgenol. 190(3):809-14, 2008
                                            18. Warmuth-Metz M et al: CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system. Neuroradiology. 50(5):447-52, 2008
                                            19. Meyers SP et al: Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am J Neuroradiol. 27(5):962-71, 2006
                                            20. Parmar H et al: Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors. Pediatr Radiol. 36(2):126-32, 2006
                                            21. Fenton LZ et al: Atypical teratoid/rhabdoid tumor of the central nervous system in children: an atypical series and review. Pediatr Radiol. 33(8):554-8, 2003
                                            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

                                                        • Synonyms

                                                          • Malignant rhabdoid tumor of brain
                                                          • Intracranial rhabdoid tumor

                                                        • Definitions

                                                          • Atypical teratoid/rhabdoid tumor (AT/RT): Malignant embryonal tumor of CNS in early childhood
                                                            • Closely related to renal & soft tissue rhabdoid tumors

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR 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. Zhang M et al: Radiomic phenotypes distinguish atypical teratoid/rhabdoid tumors from medulloblastoma. AJNR Am J Neuroradiol. 42(9):1702-8, 2021
                                                                                      2. Tamrazi B et al: Pediatric atypical teratoid/rhabdoid tumors of the brain: identification of metabolic subgroups using in vivo 1H-MR spectroscopy. AJNR Am J Neuroradiol. 40(5):872-7, 2019
                                                                                      3. Nowak J et al: Magnetic resonance imaging surrogates of molecular subgroups in atypical teratoid/rhabdoid tumor. Neuro Oncol. 20(12):1672-9, 2018
                                                                                      4. Fischer-Valuck BW et al: Assessment of the treatment approach and survival outcomes in a modern cohort of patients with atypical teratoid rhabdoid tumors using the National Cancer Database. Cancer. 123(4):682-7, 2017
                                                                                      5. Katz JS et al: Cerebellopontine angle tumors in young children, displaying cranial nerve deficits, and restricted diffusion on diffusion-weighted imaging: a new clinical triad for atypical teratoid/rhabdoid tumors. Childs Nerv Syst. 33(5):833-8, 2017
                                                                                      6. Frühwald MC et al: Atypical teratoid/rhabdoid tumors-current concepts, advances in biology, and potential future therapies. Neuro Oncol. 18(6):764-78, 2016
                                                                                      7. Johann PD et al: Atypical teratoid/rhabdoid tumors are comprised of three epigenetic subgroups with distinct enhancer landscapes. Cancer Cell. 29(3):379-93, 2016
                                                                                      8. DiPatri AJ Jr et al: Atypical teratoid rhabdoid tumors of the posterior fossa in children. Childs Nerv Syst. 31(10):1717-28, 2015
                                                                                      9. Ho DM et al: Integrated genomics has identified a new AT/RT-like yet INI1-positive brain tumor subtype among primary pediatric embryonal tumors. BMC Med Genomics. 8:32, 2015
                                                                                      10. Lau CS et al: Atypical teratoid rhabdoid tumors: a population-based clinical outcomes study involving 174 patients from the Surveillance, Epidemiology, and End Results database (1973-2010). Cancer Manag Res. 7:301-9, 2015
                                                                                      11. Torchia J et al: Molecular subgroups of atypical teratoid rhabdoid tumours in children: an integrated genomic and clinicopathological analysis. Lancet Oncol. 16(5):569-82, 2015
                                                                                      12. Ostrom QT et al: CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2007-2011. Neuro Oncol. 16 Suppl 4:iv1-63, 2014
                                                                                      13. Slavc I et al: Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012. Cancer Med. 3(1):91-100, 2014
                                                                                      14. Au Yong KJ et al: How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors? Pediatr Radiol. 43(3):347-54, 2013
                                                                                      15. Bruggers CS et al: Magnetic resonance imaging spectroscopy in pediatric atypical teratoid rhabdoid tumors of the brain. J Pediatr Hematol Oncol. 36(6):341-5, 2013
                                                                                      16. Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
                                                                                      17. Koral K et al: Imaging characteristics of atypical teratoid-rhabdoid tumor in children compared with medulloblastoma. AJR Am J Roentgenol. 190(3):809-14, 2008
                                                                                      18. Warmuth-Metz M et al: CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system. Neuroradiology. 50(5):447-52, 2008
                                                                                      19. Meyers SP et al: Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am J Neuroradiol. 27(5):962-71, 2006
                                                                                      20. Parmar H et al: Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors. Pediatr Radiol. 36(2):126-32, 2006
                                                                                      21. Fenton LZ et al: Atypical teratoid/rhabdoid tumor of the central nervous system in children: an atypical series and review. Pediatr Radiol. 33(8):554-8, 2003