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Brainstem Tumors
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Brainstem tumors (BSTs)
              • Diffuse intrinsic pontine glioma (DIPG): Diffuse midline glioma (DMG) in pons
                • Most common form of DMG in children
            • Synonyms

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

              • BSTs are distinguished by location & imaging/histologic characteristics of tumor
                • DMG in pons (classically referred to as DIPG)
                  • Glial tumor centered in pons (WHO grades 2-4)
                  • 80% of pediatric BSTs
                  • Expansile, often poorly marginated, often lacks enhancement
                  • 70-80% harbor H3 K27M mutation → poor prognosis
                • Focal pontine tumors: Embryonal tumor with multilayered rosettes (ETMR), ganglioglioma
                  • Much less common than DIPG/DMG
                • Tectal gliomas
                  • Present with hydrocephalus in 6- to 10-year-olds
                  • Rarely progressive
                  • CSF diversion is often only treatment required
                • Midbrain or mesencephalic tumors
                  • All histologies occur (including embryonal tumors & atypical teratoid/rhabdoid tumors)
                • Medullary tumors
                  • Pilocytic astrocytoma (PA) is most likely histology
                  • Usually dorsally exophytic & solidly enhancing
                • BSTs associated with neurofibromatosis type 1 (NF1)
                  • Typically PA
                  • 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. Sarma A et al: Magnetic resonance imaging of the brainstem in children, part 2: acquired pathology of the pediatric brainstem. Pediatr Radiol. 51(2):189-204, 2021
                                              2. Cooney TM et al: Response assessment in diffuse intrinsic pontine glioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) working group. Lancet Oncol. 21(6):e330-6, 2020
                                              3. Leach JL et al: MR imaging features of diffuse intrinsic pontine glioma and relationship to overall survival: report from the International DIPG Registry. Neuro Oncol. 22(11):1647-57, 2020
                                              4. Pfaff E et al: Brainstem biopsy in pediatric diffuse intrinsic pontine glioma in the era of precision medicine: the INFORM study experience. Eur J Cancer. 114:27-35, 2019
                                              5. Hoffman LM et al: Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): a collaborative report from the International and European Society for Pediatric Oncology DIPG Registries. J Clin Oncol. 36(19):1963-72, 2018
                                              6. 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):37112-24, 2018
                                              7. 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
                                              8. Castel D et al: Histone H3 genotyping refines clinico-radiological diagnostic and prognostic criteria in DIPG. Acta Neuropathol. 131(5):795-6, 2016
                                              9. Klimo P Jr et al: Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas. J Neurosurg Pediatr. 17(1):57-65, 2016
                                              10. Tisnado J et al: Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 31(12):1386-93, 2016
                                              11. Buczkowicz P et al: Pathology, molecular genetics, and epigenetics of diffuse intrinsic pontine glioma. Front Oncol. 5:147, 2015
                                              12. Gass D et al: Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae. J Neurooncol. 122(1):169-77, 2015
                                              13. 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
                                              14. 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
                                              15. 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
                                              16. 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
                                              17. 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
                                              18. 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
                                              19. Garzón M et al: Brain stem tumors in children and adolescents: single institutional experience. Childs Nerv Syst. 29(8):1321-31, 2013
                                              20. 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
                                              21. Igboechi C et al: Tectal plate gliomas: a review. Childs Nerv Syst. 29(10):1827-33, 2013
                                              22. Jackson S et al: Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma. J Neurooncol. 114(3):339-44, 2013
                                              23. Ramos A et al: Brainstem gliomas. Semin Ultrasound CT MR. 34(2):104-12, 2013
                                              24. Yin L et al: Correlation between MRI findings and histological diagnosis of brainstem glioma. Can J Neurol Sci. 40(3):348-54, 2013
                                              25. 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
                                              26. 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

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Brainstem tumors (BSTs)
                                                          • Diffuse intrinsic pontine glioma (DIPG): Diffuse midline glioma (DMG) in pons
                                                            • Most common form of DMG in children
                                                        • Synonyms

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

                                                          • BSTs are distinguished by location & imaging/histologic characteristics of tumor
                                                            • DMG in pons (classically referred to as DIPG)
                                                              • Glial tumor centered in pons (WHO grades 2-4)
                                                              • 80% of pediatric BSTs
                                                              • Expansile, often poorly marginated, often lacks enhancement
                                                              • 70-80% harbor H3 K27M mutation → poor prognosis
                                                            • Focal pontine tumors: Embryonal tumor with multilayered rosettes (ETMR), ganglioglioma
                                                              • Much less common than DIPG/DMG
                                                            • Tectal gliomas
                                                              • Present with hydrocephalus in 6- to 10-year-olds
                                                              • Rarely progressive
                                                              • CSF diversion is often only treatment required
                                                            • Midbrain or mesencephalic tumors
                                                              • All histologies occur (including embryonal tumors & atypical teratoid/rhabdoid tumors)
                                                            • Medullary tumors
                                                              • Pilocytic astrocytoma (PA) is most likely histology
                                                              • Usually dorsally exophytic & solidly enhancing
                                                            • BSTs associated with neurofibromatosis type 1 (NF1)
                                                              • Typically PA
                                                              • 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. Sarma A et al: Magnetic resonance imaging of the brainstem in children, part 2: acquired pathology of the pediatric brainstem. Pediatr Radiol. 51(2):189-204, 2021
                                                                                          2. Cooney TM et al: Response assessment in diffuse intrinsic pontine glioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) working group. Lancet Oncol. 21(6):e330-6, 2020
                                                                                          3. Leach JL et al: MR imaging features of diffuse intrinsic pontine glioma and relationship to overall survival: report from the International DIPG Registry. Neuro Oncol. 22(11):1647-57, 2020
                                                                                          4. Pfaff E et al: Brainstem biopsy in pediatric diffuse intrinsic pontine glioma in the era of precision medicine: the INFORM study experience. Eur J Cancer. 114:27-35, 2019
                                                                                          5. Hoffman LM et al: Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): a collaborative report from the International and European Society for Pediatric Oncology DIPG Registries. J Clin Oncol. 36(19):1963-72, 2018
                                                                                          6. 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):37112-24, 2018
                                                                                          7. 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
                                                                                          8. Castel D et al: Histone H3 genotyping refines clinico-radiological diagnostic and prognostic criteria in DIPG. Acta Neuropathol. 131(5):795-6, 2016
                                                                                          9. Klimo P Jr et al: Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas. J Neurosurg Pediatr. 17(1):57-65, 2016
                                                                                          10. Tisnado J et al: Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 31(12):1386-93, 2016
                                                                                          11. Buczkowicz P et al: Pathology, molecular genetics, and epigenetics of diffuse intrinsic pontine glioma. Front Oncol. 5:147, 2015
                                                                                          12. Gass D et al: Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae. J Neurooncol. 122(1):169-77, 2015
                                                                                          13. 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
                                                                                          14. 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
                                                                                          15. 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
                                                                                          16. 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
                                                                                          17. 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
                                                                                          18. 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
                                                                                          19. Garzón M et al: Brain stem tumors in children and adolescents: single institutional experience. Childs Nerv Syst. 29(8):1321-31, 2013
                                                                                          20. 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
                                                                                          21. Igboechi C et al: Tectal plate gliomas: a review. Childs Nerv Syst. 29(10):1827-33, 2013
                                                                                          22. Jackson S et al: Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma. J Neurooncol. 114(3):339-44, 2013
                                                                                          23. Ramos A et al: Brainstem gliomas. Semin Ultrasound CT MR. 34(2):104-12, 2013
                                                                                          24. Yin L et al: Correlation between MRI findings and histological diagnosis of brainstem glioma. Can J Neurol Sci. 40(3):348-54, 2013
                                                                                          25. 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
                                                                                          26. 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