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Atypical Teratoid/Rhabdoid Tumor
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Atypical teratoid/rhabdoid tumor (AT/RT)
            • Synonyms

              • Malignant rhabdoid tumor of brain
              • Rhabdoid brain tumor
            • Definitions

              • Lethal (usually childhood) cancer with SMARCB1 mutations
                • Loss of INI1 protein expression on IHC = hallmark of CNS AT/RT

            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. Bhimani AD et al: Differentiation of outcomes by treatment regimen and histology in CNS primary embryonal tumors. World Neurosurg. ePub, 2020
                                          2. Blessing MM et al: Embryonal tumors of the central nervous system: an update. Surg Pathol Clin. 13(2):235-47, 2020
                                          3. Cacciotti C et al: Advances in the molecular classification of pediatric brain tumors: a guide to the galaxy. J Pathol. ePub, 2020
                                          4. Li G et al: Screening for genes and subnetworks associated with atypical teratoid/rhabdoid tumors using bioinformatics analysis. Int J Neurosci. 1-8, 2020
                                          5. Pan X et al: Identification of hub genes in atypical teratoid/rhabdoid tumor by bioinformatics analyses. J Mol Neurosci. ePub, 2020
                                          6. Kristensen BW et al: Molecular pathology of tumors of the central nervous system. Ann Oncol. 30(1):1265-78, 2019
                                          7. Nesvick CL et al: Case-based review: atypical teratoid/rhabdoid tumor. Neurooncol Pract. 6(3):163-78, 2019
                                          8. Yang WC et al: Role of early and aggressive post-operative radiation therapy in improving outcome for pediatric central nervous system atypical teratoid/rhabdoid tumor. Childs Nerv Syst. 35(6):1013-20, 2019
                                          9. Chan V et al: A systematic review of atypical teratoid rhabdoid tumor in adults. Front Oncol. 8:567, 2018
                                          10. Takayasu T et al: Advantages of high b-value diffusion-weighted imaging for preoperative differential diagnosis between embryonal and ependymal tumors at 3T MRI. Eur J Radiol. 101:136-43, 2018
                                          11. Al-Hussaini M et al: Atypical teratoid/ rhabdoid tumor, an immunohistochemical study of potential diagnostic and prognostic markers. Neuropathology. 36(1):17-26, 2016
                                          12. Frühwald MC et al: Atypical teratoid/rhabdoid tumors-current concepts, advances in biology, and potential future therapies. Neuro Oncol. 18(6):764-78, 2016
                                          13. Sredni ST et al: Rhabdoid tumor predisposition syndrome. Pediatr Dev Pathol. 18(1):49-58, 2015
                                          14. 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
                                          15. Agaimy A: The expanding family of SMARCB1(INI1)-deficient neoplasia: implications of phenotypic, biological, and molecular heterogeneity. Adv Anat Pathol. 21(6):394-410, 2014
                                          16. Margol AS et al: Pathology and diagnosis of SMARCB1-deficient tumors. Cancer Genet. 207(9):358-64, 2014
                                          17. Souki C et al: Atypical teratoid rhabdoid tumor in adulthood. Clin Neuropathol. 33(3):245-50, 2014
                                          18. Au Yong KJ et al: How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors? Pediatr Radiol. 43(3):347-54, 2013
                                          19. Koral K et al: Diffusion MRI improves the accuracy of preoperative diagnosis of common pediatric cerebellar tumors among reviewers with different experience levels. AJNR Am J Neuroradiol. 34(12):2360-5, 2013
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Atypical teratoid/rhabdoid tumor (AT/RT)
                                                    • Synonyms

                                                      • Malignant rhabdoid tumor of brain
                                                      • Rhabdoid brain tumor
                                                    • Definitions

                                                      • Lethal (usually childhood) cancer with SMARCB1 mutations
                                                        • Loss of INI1 protein expression on IHC = hallmark of CNS AT/RT

                                                    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. Bhimani AD et al: Differentiation of outcomes by treatment regimen and histology in CNS primary embryonal tumors. World Neurosurg. ePub, 2020
                                                                                  2. Blessing MM et al: Embryonal tumors of the central nervous system: an update. Surg Pathol Clin. 13(2):235-47, 2020
                                                                                  3. Cacciotti C et al: Advances in the molecular classification of pediatric brain tumors: a guide to the galaxy. J Pathol. ePub, 2020
                                                                                  4. Li G et al: Screening for genes and subnetworks associated with atypical teratoid/rhabdoid tumors using bioinformatics analysis. Int J Neurosci. 1-8, 2020
                                                                                  5. Pan X et al: Identification of hub genes in atypical teratoid/rhabdoid tumor by bioinformatics analyses. J Mol Neurosci. ePub, 2020
                                                                                  6. Kristensen BW et al: Molecular pathology of tumors of the central nervous system. Ann Oncol. 30(1):1265-78, 2019
                                                                                  7. Nesvick CL et al: Case-based review: atypical teratoid/rhabdoid tumor. Neurooncol Pract. 6(3):163-78, 2019
                                                                                  8. Yang WC et al: Role of early and aggressive post-operative radiation therapy in improving outcome for pediatric central nervous system atypical teratoid/rhabdoid tumor. Childs Nerv Syst. 35(6):1013-20, 2019
                                                                                  9. Chan V et al: A systematic review of atypical teratoid rhabdoid tumor in adults. Front Oncol. 8:567, 2018
                                                                                  10. Takayasu T et al: Advantages of high b-value diffusion-weighted imaging for preoperative differential diagnosis between embryonal and ependymal tumors at 3T MRI. Eur J Radiol. 101:136-43, 2018
                                                                                  11. Al-Hussaini M et al: Atypical teratoid/ rhabdoid tumor, an immunohistochemical study of potential diagnostic and prognostic markers. Neuropathology. 36(1):17-26, 2016
                                                                                  12. Frühwald MC et al: Atypical teratoid/rhabdoid tumors-current concepts, advances in biology, and potential future therapies. Neuro Oncol. 18(6):764-78, 2016
                                                                                  13. Sredni ST et al: Rhabdoid tumor predisposition syndrome. Pediatr Dev Pathol. 18(1):49-58, 2015
                                                                                  14. 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
                                                                                  15. Agaimy A: The expanding family of SMARCB1(INI1)-deficient neoplasia: implications of phenotypic, biological, and molecular heterogeneity. Adv Anat Pathol. 21(6):394-410, 2014
                                                                                  16. Margol AS et al: Pathology and diagnosis of SMARCB1-deficient tumors. Cancer Genet. 207(9):358-64, 2014
                                                                                  17. Souki C et al: Atypical teratoid rhabdoid tumor in adulthood. Clin Neuropathol. 33(3):245-50, 2014
                                                                                  18. Au Yong KJ et al: How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors? Pediatr Radiol. 43(3):347-54, 2013
                                                                                  19. Koral K et al: Diffusion MRI improves the accuracy of preoperative diagnosis of common pediatric cerebellar tumors among reviewers with different experience levels. AJNR Am J Neuroradiol. 34(12):2360-5, 2013