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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
- DIPG: Glial tumor centered in pons (WHO grades II-IV)
- BST distinguished by location & imaging/histologic characteristics of tumor
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
- 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
- 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
- Castel D et al: Histone H3 genotyping refines clinico-radiological diagnostic and prognostic criteria in DIPG. Acta Neuropathol. 131(5):795-6, 2016
- Klimo P Jr et al: Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas. J Neurosurg Pediatr. 17(1):57-65, 2016
- Tisnado J et al: Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 31(12):1386-93, 2016
- Buczkowicz P et al: Pathology, molecular genetics, and epigenetics of diffuse intrinsic pontine glioma. Front Oncol. 5:147, 2015
- Gass D et al: Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae. J Neurooncol. 122(1):169-77, 2015
- 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
- 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
- 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
- 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
- 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
- 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
- Garzón M et al: Brain stem tumors in children and adolescents: single institutional experience. Childs Nerv Syst. 29(8):1321-31, 2013
- 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
- Igboechi C et al: Tectal plate gliomas: a review. Childs Nerv Syst. 29(10):1827-33, 2013
- Jackson S et al: Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma. J Neurooncol. 114(3):339-44, 2013
- Ramos A et al: Brainstem gliomas. Semin Ultrasound CT MR. 34(2):104-12, 2013
- Yin L et al: Correlation between MRI findings and histological diagnosis of brainstem glioma. Can J Neurol Sci. 40(3):348-54, 2013
- 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
- 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
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References
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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
- DIPG: Glial tumor centered in pons (WHO grades II-IV)
- BST distinguished by location & imaging/histologic characteristics of tumor
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
- 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
- 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
- Castel D et al: Histone H3 genotyping refines clinico-radiological diagnostic and prognostic criteria in DIPG. Acta Neuropathol. 131(5):795-6, 2016
- Klimo P Jr et al: Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas. J Neurosurg Pediatr. 17(1):57-65, 2016
- Tisnado J et al: Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 31(12):1386-93, 2016
- Buczkowicz P et al: Pathology, molecular genetics, and epigenetics of diffuse intrinsic pontine glioma. Front Oncol. 5:147, 2015
- Gass D et al: Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae. J Neurooncol. 122(1):169-77, 2015
- 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
- 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
- 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
- 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
- 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
- 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
- Garzón M et al: Brain stem tumors in children and adolescents: single institutional experience. Childs Nerv Syst. 29(8):1321-31, 2013
- 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
- Igboechi C et al: Tectal plate gliomas: a review. Childs Nerv Syst. 29(10):1827-33, 2013
- Jackson S et al: Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma. J Neurooncol. 114(3):339-44, 2013
- Ramos A et al: Brainstem gliomas. Semin Ultrasound CT MR. 34(2):104-12, 2013
- Yin L et al: Correlation between MRI findings and histological diagnosis of brainstem glioma. Can J Neurol Sci. 40(3):348-54, 2013
- 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
- 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
