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Brainstem Tumors
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Brainstem tumors (BSTs), diffuse intrinsic pontine glioma (DIPG)
              • Synonyms

                • Pontine glioma, midbrain glioma, medullary glioma, dorsally exophytic medullary glioma
              • Definitions

                • BST distinguished by location & imaging/histologic characteristics of tumor
                  • DIPG: Glial tumor centered in pons (WHO grades II-IV)
                    • Expansile, often poorly marginated, often lack enhancement
                    • Glioblastoma multiforme (GBM) shows focal enhancement & central necrosis
                  • Tectal gliomas
                    • Present with hydrocephalus in 6-10 year olds
                    • Rarely progressive
                    • CSF diversion often only treatment required
                  • Midbrain or mesencephalic tumors
                    • All histologies occur (including PNET, atypical teratoid rhabdoid tumor)
                  • Medullary tumors
                    • PA most likely histology
                    • Usually dorsally exophytic & solidly enhancing
                  • BST associated with neurofibromatosis type 1 (NF1) → another distinct group
                    • Rarely enlarge, often asymptomatic

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine 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

                                              • Reporting Tips

                                                Selected References

                                                1. Huang T et al: Detection of histone H3 K27M mutation and post-translational modifications in pediatric diffuse midline glioma via tissue immunohistochemistry informs diagnosis and clinical outcomes. Oncotarget. 9(98):37112-37124, 2018
                                                2. Hamisch C et al: Update on the diagnostic value and safety of stereotactic biopsy for pediatric brainstem tumors: a systematic review and meta-analysis of 735 cases. J Neurosurg Pediatr. 20(3):261-8, 2017
                                                3. Castel D et al: Histone H3 genotyping refines clinico-radiological diagnostic and prognostic criteria in DIPG. Acta Neuropathol. 131(5):795-6, 2016
                                                4. Klimo P Jr et al: Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas. J Neurosurg Pediatr. 17(1):57-65, 2016
                                                5. Tisnado J et al: Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 31(12):1386-93, 2016
                                                6. Buczkowicz P et al: Pathology, molecular genetics, and epigenetics of diffuse intrinsic pontine glioma. Front Oncol. 5:147, 2015
                                                7. Gass D et al: Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae. J Neurooncol. 122(1):169-77, 2015
                                                8. Jansen MH et al: Survival prediction model of children with diffuse intrinsic pontine glioma based on clinical and radiological criteria. Neuro Oncol. 17(1):160-6, 2015
                                                9. Poussaint TY et al: Apparent diffusion coefficient histogram metrics correlate with survival in diffuse intrinsic pontine glioma: a report from the Pediatric Brain Tumor Consortium. Neuro Oncol. 18(5):725-34, 2015
                                                10. Griessenauer CJ et al: Pediatric tectal plate gliomas: clinical and radiological progression, MR imaging characteristics, and management of hydrocephalus. J Neurosurg Pediatr. 13(1):13-20, 2014
                                                11. Lober RM et al: Diffusion-weighted MRI derived apparent diffusion coefficient identifies prognostically distinct subgroups of pediatric diffuse intrinsic pontine glioma. J Neurooncol. 117(1):175-82, 2014
                                                12. Lundar T et al: Neurosurgical treatment of pediatric low-grade midbrain tumors: a single consecutive institutional series of 15 patients. J Neurosurg Pediatr. 14(6):598-603, 2014
                                                13. Cage TA et al: Feasibility, safety, and indications for surgical biopsy of intrinsic brainstem tumors in children. Childs Nerv Syst. 29(8):1313-9, 2013
                                                14. Garzón M et al: Brain stem tumors in children and adolescents: single institutional experience. Childs Nerv Syst. 29(8):1321-31, 2013
                                                15. Goda JS et al: Can multiparametric MRI and FDG-PET predict outcome in diffuse brainstem glioma? A report from a prospective phase-II study. Pediatr Neurosurg. 49(5):274-81, 2013
                                                16. Igboechi C et al: Tectal plate gliomas: a review. Childs Nerv Syst. 29(10):1827-33, 2013
                                                17. Jackson S et al: Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma. J Neurooncol. 114(3):339-44, 2013
                                                18. Ramos A et al: Brainstem gliomas. Semin Ultrasound CT MR. 34(2):104-12, 2013
                                                19. Yin L et al: Correlation between MRI findings and histological diagnosis of brainstem glioma. Can J Neurol Sci. 40(3):348-54, 2013
                                                20. Laprie A et al: Longitudinal multivoxel MR spectroscopy study of pediatric diffuse brainstem gliomas treated with radiotherapy. Int J Radiat Oncol Biol Phys. 62(1):20-31, 2005
                                                21. Broniscer A et al: Brain stem involvement in children with neurofibromatosis type 1: role of magnetic resonance imaging and spectroscopy in the distinction from diffuse pontine glioma. Neurosurgery. 40(2):331-7; discussion 337-8, 1997
                                                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

                                                              • Brainstem tumors (BSTs), diffuse intrinsic pontine glioma (DIPG)
                                                            • Synonyms

                                                              • Pontine glioma, midbrain glioma, medullary glioma, dorsally exophytic medullary glioma
                                                            • Definitions

                                                              • BST distinguished by location & imaging/histologic characteristics of tumor
                                                                • DIPG: Glial tumor centered in pons (WHO grades II-IV)
                                                                  • Expansile, often poorly marginated, often lack enhancement
                                                                  • Glioblastoma multiforme (GBM) shows focal enhancement & central necrosis
                                                                • Tectal gliomas
                                                                  • Present with hydrocephalus in 6-10 year olds
                                                                  • Rarely progressive
                                                                  • CSF diversion often only treatment required
                                                                • Midbrain or mesencephalic tumors
                                                                  • All histologies occur (including PNET, atypical teratoid rhabdoid tumor)
                                                                • Medullary tumors
                                                                  • PA most likely histology
                                                                  • Usually dorsally exophytic & solidly enhancing
                                                                • BST associated with neurofibromatosis type 1 (NF1) → another distinct group
                                                                  • Rarely enlarge, often asymptomatic

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Nuclear Medicine 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

                                                                                            • Reporting Tips

                                                                                              Selected References

                                                                                              1. Huang T et al: Detection of histone H3 K27M mutation and post-translational modifications in pediatric diffuse midline glioma via tissue immunohistochemistry informs diagnosis and clinical outcomes. Oncotarget. 9(98):37112-37124, 2018
                                                                                              2. Hamisch C et al: Update on the diagnostic value and safety of stereotactic biopsy for pediatric brainstem tumors: a systematic review and meta-analysis of 735 cases. J Neurosurg Pediatr. 20(3):261-8, 2017
                                                                                              3. Castel D et al: Histone H3 genotyping refines clinico-radiological diagnostic and prognostic criteria in DIPG. Acta Neuropathol. 131(5):795-6, 2016
                                                                                              4. Klimo P Jr et al: Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas. J Neurosurg Pediatr. 17(1):57-65, 2016
                                                                                              5. Tisnado J et al: Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 31(12):1386-93, 2016
                                                                                              6. Buczkowicz P et al: Pathology, molecular genetics, and epigenetics of diffuse intrinsic pontine glioma. Front Oncol. 5:147, 2015
                                                                                              7. Gass D et al: Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae. J Neurooncol. 122(1):169-77, 2015
                                                                                              8. Jansen MH et al: Survival prediction model of children with diffuse intrinsic pontine glioma based on clinical and radiological criteria. Neuro Oncol. 17(1):160-6, 2015
                                                                                              9. Poussaint TY et al: Apparent diffusion coefficient histogram metrics correlate with survival in diffuse intrinsic pontine glioma: a report from the Pediatric Brain Tumor Consortium. Neuro Oncol. 18(5):725-34, 2015
                                                                                              10. Griessenauer CJ et al: Pediatric tectal plate gliomas: clinical and radiological progression, MR imaging characteristics, and management of hydrocephalus. J Neurosurg Pediatr. 13(1):13-20, 2014
                                                                                              11. Lober RM et al: Diffusion-weighted MRI derived apparent diffusion coefficient identifies prognostically distinct subgroups of pediatric diffuse intrinsic pontine glioma. J Neurooncol. 117(1):175-82, 2014
                                                                                              12. Lundar T et al: Neurosurgical treatment of pediatric low-grade midbrain tumors: a single consecutive institutional series of 15 patients. J Neurosurg Pediatr. 14(6):598-603, 2014
                                                                                              13. Cage TA et al: Feasibility, safety, and indications for surgical biopsy of intrinsic brainstem tumors in children. Childs Nerv Syst. 29(8):1313-9, 2013
                                                                                              14. Garzón M et al: Brain stem tumors in children and adolescents: single institutional experience. Childs Nerv Syst. 29(8):1321-31, 2013
                                                                                              15. Goda JS et al: Can multiparametric MRI and FDG-PET predict outcome in diffuse brainstem glioma? A report from a prospective phase-II study. Pediatr Neurosurg. 49(5):274-81, 2013
                                                                                              16. Igboechi C et al: Tectal plate gliomas: a review. Childs Nerv Syst. 29(10):1827-33, 2013
                                                                                              17. Jackson S et al: Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma. J Neurooncol. 114(3):339-44, 2013
                                                                                              18. Ramos A et al: Brainstem gliomas. Semin Ultrasound CT MR. 34(2):104-12, 2013
                                                                                              19. Yin L et al: Correlation between MRI findings and histological diagnosis of brainstem glioma. Can J Neurol Sci. 40(3):348-54, 2013
                                                                                              20. Laprie A et al: Longitudinal multivoxel MR spectroscopy study of pediatric diffuse brainstem gliomas treated with radiotherapy. Int J Radiat Oncol Biol Phys. 62(1):20-31, 2005
                                                                                              21. Broniscer A et al: Brain stem involvement in children with neurofibromatosis type 1: role of magnetic resonance imaging and spectroscopy in the distinction from diffuse pontine glioma. Neurosurgery. 40(2):331-7; discussion 337-8, 1997