多倫多病童醫(yī)院腦干膠質(zhì)瘤ppt課件

1、among brainstem gliomasAtectal gliomaBfocal midbrain tumorCfocal intrinsic pontine gliomaDdorsal/exophytic gliomaEdiffuse intrinsic pontine glioma*Ffocal medullary gliomaGcervicomedullary gliomaA Few Important Distinctions* a form of high grade glioma, akin toanaplastic astrocytoma or glioblastoma m

2、ultiformeAugust 2001August 2006October 2021August 2000December 2001December 2001December 2002Low grade glioma of the brainstem: chemotherapy with weekly vincristine and carboplatinDiagnosis (11/2021)1/2021 (one year of VBL)BRAF V600 mutated tumourTypical DPGTypical BSG 13 year old10 month history of

3、 progressive right sided weakness, (R) CN 7 and 8Grade 2 on histolology 17 year old12 month history of dizziness when lying downNo CN deficit, no Long tract sign, no ataxia11 year-oldJanuary 20042021 (18 years old)January 200420211 day oldPM: PNET1 day oldNo PM4 month oldPilocytic AstrocytomaOn chem

4、oDPGLGGFocal HGGDPGLGG2 year-old, 5 months history of ataxia and gaze palsyBiopsy: low grade astrocytoma3 years old, NF110/20217/20213 years old Mild hemiparesisBiopsy: infiltrative astrocytoma (grade 2)9/202110/2021MALIGNANT GLIOMA OF PONSCANADIAN CASES BY YEAR Standard RT50-54 Gy in 1.8 GyDaily fr

5、actionsCurrent trend to move to conformal techniquesHyperfractionation: results of prospective studiesFreeman et al, POG 9239, IJROBP199954 Gy in 30 fractions versus 39 Gy in 13 fractionsZhagloul et alRadiotherapy & Oncology 2021PATIENT DIED AT 11 MONTHS POST DIAGNOSISAge at Diagnosis(MONTHS)SexNeur

6、ological Signs at PresentationInterval Between Onset of Symptoms and Diagnosis(Weeks)Initial TreatmentSurvival (Years)Cranial Nerve PalsyPyramidal DeficitsCerebellar Signs20MaleYesYesYes24 RT +Temozolomide+522MaleYesYesNo12-24 RT+4CLINICAL CHARACTERISTICS, TREATMENT AND OUTCOME OF SURVIVING PATIENTS

7、MRI IMAGING OF LONG TERM SURVIVORSOctober 2021January 2021January 2021Long term survivorArsenic trioxyde (antivascular effect, radiosensitizer)BiopsyCohort 1 MGMT- EGFR-Cohort 2 MGMT- EGFR+Cohort 3 MGMT+ EGFR-Cohort 4 MGMT+EGFR+RT BevacizumabRTBevacizumab ErlotinibRTBevacizumabTemozolomideRTBevacizu

8、mab ErlotinibTemozolomide4 Weeks Bevacizumab4 Weeks Bevacizumab Erlotinib4 Weeks Bevacizumab4 Weeks Bevacizumab ErlotinibMaintenance BevacizumabMaintenance Bevacizumab ErlotinibMaintenanceBevacizumab TemozolomideMaintenance Bevacizumab ErlotinibTemozolomideMRI Diagnosis DIPGTREATMENT SCHEMAEnrollmen

9、tTissue AnalysesBoston/UCSF protocolLeft facial nerve weaknessDisconjugate gazeWeakness bilateral 6th nerves (left greater than right)Gait discoordinationDec 2021Oct 202130 Gy in 17 sessions Oct 2021: 54 Gy in 30 sessions DIPGsHGAs13579112468101315171921X12141618202213579111315171921X246810121416182

10、022DIPGs are genetically distinct from supratentorial high grade astrocytomasDIPGHGA12345678910111234567891011Chromosome 14Chromosome 17p13p12p11.2q11.1q11.2q12q13.1q21.1q21.2q21.3q23.1q22.1q23.2q23.3q24.1q24.2q24.3q31.1q31.3q32.13q32.2q32.33p13.3p13.2p13.1p11.2p12q11.2q12q21.2q21.31q21.32q21.33q22q23.2q24.1q24.2q24.3q25.1q25.3p13p12p11.2q11.1q11.2q12q13.1q21.1q21.2q21.3q23.1q22.1q23.2q23.3q24.1q24.2q24.3q31.1q31.3q32.13q32.2q32.33p13.3p13.2p13.1p11.2p12q11.2q12q21.2q21.31q21.32q21.33q22q23.2q24.1q24.2q24.3q25.1q25.3DIPGs are genetically distinct from suprate