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Atypical Teratoid-Rhabdoid Tumor
Anne G. Osborn, MD, FACR; Chang Yueh Ho, MD
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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/hSNF5 mutations
                • Loss of INI1 protein expression 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. Sredni ST et al: Rhabdoid tumor predisposition syndrome. Pediatr Dev Pathol. 18(1):49-58, 2015
                                          2. 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
                                          3. 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
                                          4. Kubicky CD et al: Rare primary central nervous system tumors. Rare Tumors. 6(3):5449, 2014
                                          5. Louis DN et al: International Society Of Neuropathology--Haarlem consensus guidelines for nervous system tumor classification and grading. Brain Pathol. 24(5):429-35, 2014
                                          6. Margol AS et al: Pathology and diagnosis of SMARCB1-deficient tumors. Cancer Genet. 207(9):358-64, 2014
                                          7. Siu A et al: Association of cerebellopontine angle atypical teratoid/rhabdoid tumors with acute facial nerve palsy in infants. J Neurosurg Pediatr. 13(1):29-32, 2014
                                          8. Souki C et al: Atypical teratoid rhabdoid tumor in adulthood. Clin Neuropathol. 33(3):245-50, 2014
                                          9. Xin X et al: A primary spinal extradural atypical teratoid/rhabdoid tumor of the cervical spine with bony involvement. J Child Neurol. 29(5):670-3, 2014
                                          10. Au Yong KJ et al: How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors? Pediatr Radiol. 43(3):347-54, 2013
                                          11. Bruggers CS et al: Magnetic resonance imaging spectroscopy in pediatric atypical teratoid rhabdoid tumors of the brain. J Pediatr Hematol Oncol. Epub ahead of print, 2013
                                          12. 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
                                          13. Birks DK et al: Claudin 6 is a positive marker for atypical teratoid/rhabdoid tumors. Brain Pathol. 20(1):140-50, 2010
                                          14. Chi SN et al: Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol. 27(3):385-9, 2009
                                          15. 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
                                          16. 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
                                          17. Squire SE et al: Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy. J Neurooncol. 81(1):97-111, 2007
                                          18. 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
                                          19. Parmar H et al: Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors. Pediatr Radiol. 36(2):126-32, 2006
                                          20. 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

                                                  • 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/hSNF5 mutations
                                                        • Loss of INI1 protein expression 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. Sredni ST et al: Rhabdoid tumor predisposition syndrome. Pediatr Dev Pathol. 18(1):49-58, 2015
                                                                                  2. 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
                                                                                  3. 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
                                                                                  4. Kubicky CD et al: Rare primary central nervous system tumors. Rare Tumors. 6(3):5449, 2014
                                                                                  5. Louis DN et al: International Society Of Neuropathology--Haarlem consensus guidelines for nervous system tumor classification and grading. Brain Pathol. 24(5):429-35, 2014
                                                                                  6. Margol AS et al: Pathology and diagnosis of SMARCB1-deficient tumors. Cancer Genet. 207(9):358-64, 2014
                                                                                  7. Siu A et al: Association of cerebellopontine angle atypical teratoid/rhabdoid tumors with acute facial nerve palsy in infants. J Neurosurg Pediatr. 13(1):29-32, 2014
                                                                                  8. Souki C et al: Atypical teratoid rhabdoid tumor in adulthood. Clin Neuropathol. 33(3):245-50, 2014
                                                                                  9. Xin X et al: A primary spinal extradural atypical teratoid/rhabdoid tumor of the cervical spine with bony involvement. J Child Neurol. 29(5):670-3, 2014
                                                                                  10. Au Yong KJ et al: How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors? Pediatr Radiol. 43(3):347-54, 2013
                                                                                  11. Bruggers CS et al: Magnetic resonance imaging spectroscopy in pediatric atypical teratoid rhabdoid tumors of the brain. J Pediatr Hematol Oncol. Epub ahead of print, 2013
                                                                                  12. 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
                                                                                  13. Birks DK et al: Claudin 6 is a positive marker for atypical teratoid/rhabdoid tumors. Brain Pathol. 20(1):140-50, 2010
                                                                                  14. Chi SN et al: Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol. 27(3):385-9, 2009
                                                                                  15. 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
                                                                                  16. 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
                                                                                  17. Squire SE et al: Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy. J Neurooncol. 81(1):97-111, 2007
                                                                                  18. 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
                                                                                  19. Parmar H et al: Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors. Pediatr Radiol. 36(2):126-32, 2006
                                                                                  20. 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