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Medulloblastoma, Histologically-Defined
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Medulloblastoma (MB), brain tumor (BT)
              • Definitions

                • Most common malignant BT in children
                  • 2nd overall to leukemia but much worse overall prognosis
                • Malignant, invasive, highly cellular embryonal tumor of cerebellum
                  • Not single-disease entity
                    • Group of tumors with variable locations, molecular features, risk stratification, patterns of recurrence, outcome
                  • 4 distinct MB subgroups defined by methylome or gene expression profiling
                    • Wingless (WNT) activated
                    • Sonic hedgehog (SHH) activated
                    • Group 3 (Grp3)
                    • Group 4 (Grp4)
                  • Each subgroup also has additional subtypes
                    • Subtyping permits more granular view of outcome, dissemination risk profiles
                    • 12 unique subtypes, each with distinctive molecular and clinical characteristics
                    • Subgroup affiliation maintained in relapse/recurrence

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Angiographic 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

                                              Selected References

                                              1. Bhattacharya D et al: Epigenetics and survivorship in pediatric brain tumor patients. J Neurooncol. 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. Dong J et al: Differentiation between ependymoma and medulloblastoma in children with radiomics approach. Acad Radiol. ePub, 2020
                                              5. Hovestadt V et al: Medulloblastomics revisited: biological and clinical insights from thousands of patients. Nat Rev Cancer. 20(1):42-56, 2020
                                              6. Kumar R et al: Medulloblastoma genomics in the modern molecular era. Brain Pathol. 30(3):679-90, 2020
                                              7. Malbari F et al: Genetics of common pediatric brain tumors. Pediatr Neurol. 104:3-12, 2020
                                              8. Roussel MF et al: Modeling pediatric medulloblastoma. Brain Pathol. 30(3):703-12, 2020
                                              9. Van Ommeren R et al: The molecular biology of medulloblastoma metastasis. Brain Pathol. 30(3):691-702, 2020
                                              10. Zou H et al: Molecular heterogeneity and cellular diversity: implications for precision treatment in medulloblastoma. Cancers (Basel). 12(3):643, 2020
                                              11. Stock A et al: Imaging characteristics of wingless pathway subgroup medulloblastomas: Results from the German HIT/SIOP-trial cohort. AJNR Am J Neuroradiol. 40(11):1811-7, 2019
                                              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

                                                          • Abbreviations

                                                            • Medulloblastoma (MB), brain tumor (BT)
                                                          • Definitions

                                                            • Most common malignant BT in children
                                                              • 2nd overall to leukemia but much worse overall prognosis
                                                            • Malignant, invasive, highly cellular embryonal tumor of cerebellum
                                                              • Not single-disease entity
                                                                • Group of tumors with variable locations, molecular features, risk stratification, patterns of recurrence, outcome
                                                              • 4 distinct MB subgroups defined by methylome or gene expression profiling
                                                                • Wingless (WNT) activated
                                                                • Sonic hedgehog (SHH) activated
                                                                • Group 3 (Grp3)
                                                                • Group 4 (Grp4)
                                                              • Each subgroup also has additional subtypes
                                                                • Subtyping permits more granular view of outcome, dissemination risk profiles
                                                                • 12 unique subtypes, each with distinctive molecular and clinical characteristics
                                                                • Subgroup affiliation maintained in relapse/recurrence

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Angiographic 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

                                                                                          Selected References

                                                                                          1. Bhattacharya D et al: Epigenetics and survivorship in pediatric brain tumor patients. J Neurooncol. 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. Dong J et al: Differentiation between ependymoma and medulloblastoma in children with radiomics approach. Acad Radiol. ePub, 2020
                                                                                          5. Hovestadt V et al: Medulloblastomics revisited: biological and clinical insights from thousands of patients. Nat Rev Cancer. 20(1):42-56, 2020
                                                                                          6. Kumar R et al: Medulloblastoma genomics in the modern molecular era. Brain Pathol. 30(3):679-90, 2020
                                                                                          7. Malbari F et al: Genetics of common pediatric brain tumors. Pediatr Neurol. 104:3-12, 2020
                                                                                          8. Roussel MF et al: Modeling pediatric medulloblastoma. Brain Pathol. 30(3):703-12, 2020
                                                                                          9. Van Ommeren R et al: The molecular biology of medulloblastoma metastasis. Brain Pathol. 30(3):691-702, 2020
                                                                                          10. Zou H et al: Molecular heterogeneity and cellular diversity: implications for precision treatment in medulloblastoma. Cancers (Basel). 12(3):643, 2020
                                                                                          11. Stock A et al: Imaging characteristics of wingless pathway subgroup medulloblastomas: Results from the German HIT/SIOP-trial cohort. AJNR Am J Neuroradiol. 40(11):1811-7, 2019